UNIVERSITY OF THE CORDILLERAS

College of Nursing
Governor Pack Road, Baguio City, Philippines 2600
(+6374) 442-3316, 442-2564, 442-8219, 442-8256
E- mail: [email protected]
Website: www.bcf.edu.ph

CYSTIC FIBROSIS

A Case Presented to the

College of Nursing

In Partial Fulfillment of the requirements in the Course

Nursing Care Management 118

Submitted By:

ALUAD, Rosella G.
CALIXTO, Loraine Ruth P.
DULAWAN, Juliene Grace Talacca G.
GAMONNAC, Maria Crystal Quinn F.
LANGBAYAN, Zaskhia C.
LAO-ING, Jaimelyn S.
LUPAE, Frannie D.
MAGAT, Camil Joy R.
MATEO, Maria Diana A.
PIT-OG, Jhayrelle Annesy I.
REIJNDERS, Hazel Joy B.

20-NOVEMBER-2021

Noted and Approved for Presentation:

Christian Basatan & April Anne Bocato

____________________________
Signature of Adviser / Date
 ABSTRACT

TITLE: Cystic Fibrosis

AUTHOR INFORMATION: University of the Cordilleras College of Nursing 4th Year, Section C, Group 2E
– Rosella G. Aluad, Loraine Ruth P. Calixto, Juliene Grace Talacca G. Dulawan, Maria Crystal Quinn F.
Gamonnac, Zaskhia C. Langbayan, Jaimelyn S. Lao-ing, Frannie D. Lupae, Camil Joy R. Magat, Maria Diana
A. Mateo, Jhayrelle Annesy I. Pit-og, Hazel Joy B. Reijnders

BACKGROUND: Rare diseases have limited scientific data and general information often causing a delay in
seeking appropriate medical attention, obtaining proper diagnosis and appropriate treatment. The rare
occurrence of this disease have somewhat created a blind spot as compared to management of common
diseases with well-established treatment regimens.

CASE DESCRIPTION: According to Cystic Fibrosis Foundation Patient Registry, in the US, more than
30,000 people are living this disease (more than 70,000 people worldwide). It is under-diagnosed in Asia and
Africa, where infants are not tested at birth and national registries lack data (Cystic Fibrosis Foundation, 2020).
In countries without a national CF registry, most studies are conducted by individuals rather than national
organizations, making it difficult to establish a reliable prevalence.

Common to CF clients are frequent lung infections and difficulty breathing. The client has been in and out of
the hospital and is highly dependent on medications, and oxygen delivered through nasal cannula. Standard
infection precautions have been observed among everyone concerned with her care, including the family and
other patients around.

CONCLUSION: Cystic fibrosis is a rare condition requiring life-long medical management. Nursing is a
profession that entails holistic care. It focuses on every aspect: support systems, helping the patient and
families understand the condition and how they may cope. This case presentation, as much as the movie, “Five
Feet Apart” aims for CF awareness.

As future nurses, looking into this particular case reminds us the very possibility of encountering numerous
different diseases and medical cases, may it be common or rare. It is essential that we delve into complex
diseases and familiarize ourselves with as much disease as possible, including its prognosis, diagnosis, and
treatment. In this way, we are equipped and prepared with knowledge as to which nursing interventions are
necessary when faced with patients with this particular disease.

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 TABLE OF CONTENTS

I. Introduction 3
II. Statement of Objectives 4
A. General Objectives 4
B. Specific Objectives 4
III. Patient’s Profile 4
IV. Chief Complaint 4
V. Present History of Illness 4
VI. Past History of Illness 5
VII. Family Health History 5
VIII. Developmental History 5
IX. Social and Environmental History 5
X. Lifestyle and Health Practices 6
XI. Health Assessment 6
A. General Survey 6
B. Head to Toe Assessment 6
C. 13 Areas of Assessment 7
XII. Diagnostics 11
XIII. Comprehensive Pathophysiology 15
XIV. Treatment/Management 16
A. Drugs 16
XV. Nursing Care Plans 26
A. Prioritization of Problems 26
a.1. List of Problems 26
a.2. Basis for Prioritization 26
B. Nursing Care Plans 27
NCP 1: Ineffective Airway Clearance related to retained secretions 27
NCP 2: Impaired Gas Exchange related to decrease surface area for gas exchange and loss of lung
function as evidenced by crackles on both lungs and shortness of breath. 30
NCP 3: Imbalanced Nutrition: Less than body requirements related to chronic malabsorption as evidenced
by weight is 20% below the ideal body weight. 35
NCP 4: Risk for Aspiration related to the presence of gastrointestinal tube 40
NCP 5: Risk for Infection related to damage to the body’s defenses and presence of mucus as a medium
for growth of an organism. 42
C. Discharge Plan 46
XVI. Learning Insights 47
XVII. List of References 50
XVIII. Appendices 52
Appendix A 53

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I. Introduction
Harrison's Principles of Internal Medicine defines cystic fibrosis (CF) as an autosomal recessive
exocrinopathy affecting multiple epithelial tissues. It went on discussing that the gene product responsible for
CF (the cystic fibrosis transmembrane conductance regulator [CFTR]) serves as an anion channel in the apical
(luminal) plasma membranes of epithelial cells and regulates volume and composition of exocrine secretion.
Thus, the major morbidity and mortality associated with CF is attributable to respiratory compromise,
characterized by copious hyper viscous and adherent pulmonary secretions that obstruct small and medium-
sized airways. CF airway secretions are exceedingly difficult to clear, and a complex bacterial flora that
includes Staphylococcus aureus, Haemophilus influenzae, and Pseudomonas aeruginosa (among other
pathogens) is routinely cultured from CF sputum.
Robust pulmonary inflammation in the setting of inspissated (thickened) mucus and chronic bacterial
infection leads to collateral tissue injury and further aggravates respiratory decline. Organisms such as P.
aeruginosa exhibit a stereotypic mode of pathogenesis; a sentinel and early colonization event often engenders
lifelong pulmonary infection by the same genetic strain. Over the course of many years, P. aeruginosa evolves
in CF lungs to adopt a mucoid phenotype (attributable to release of alginate exoproduct) that confers selective
advantage for the pathogen and poor prognosis for the host. Infection with other bacterial organisms such as
Burkholderia cepacia also indicates a less favorable pulmonary outlook. Strategies to eradicate organisms such
as P. aeruginosa early in the pathogenesis cascade have been successful and are thought to improve prognosis
significantly if sustained.
The complete name of the disease, cystic fibrosis of the pancreas, refers to profound tissue destruction
of the exocrine pancreas, with fibrotic scarring and/or fatty replacement, cyst proliferation, loss of acinar
tissue, and ablation of normal pancreatic architecture. As in the lung, tenacious exocrine secretions (sometimes
termed concretions) obstruct pancreatic ducts and impair production and flow of digestive enzymes to the
duodenum. The sequelae of exocrine pancreatic insufficiency include chronic malabsorption, poor growth, fat-
soluble vitamin insufficiency, high levels of serum immunoreactive trypsinogen (a diagnostic test used in
newborn screening), and loss of pancreatic islet cell mass. CF-related diabetes mellitus is a manifestation in
over 30% of adults with the disease and is likely multifactorial in nature (attributable to progressive destruction
of the endocrine pancreas, insulin resistance due to stress hormones, and other factors).
Furthermore, most resources both from textbooks and online sources have listed the following as the
usual signs and symptoms associated with Cystic Fibrosis:
A. Newborn-Infant-Toddler
 Difficulty passing their first bowel movement (meconium) in newborns
 Salty-tasting sweat
 Malnutrition, difficulty gaining weight
 Bulky, bad-smelling, and greasy stool
 Frequent lung (respiratory) infections
 Coughing and wheezing
B. School Aged-Teenage-Adulthood
 Growth delays often continue
 Shortness of breath and difficulty with exercise
 Persistent coughing or wheezing accompanied by frequent chest and sinus infections with
recurring pneumonia or bronchitis
 very thick phlegm (sputum)
 Nasal polyps
 Severe or chronic sinusitis
 Pancreatitis
 Clubbing (enlargement or rounding) of the fingertips and toes
 Children (usually older than age four) sometimes have intussusceptions.
 Frequent coughing or hard-to-pass stools can occasionally cause rectal prolapse

The age at which symptoms first appear varies. Some people with cystic fibrosis were diagnosed as
babies, while others are not diagnosed until they are older. If the disease is mild at first, a person with cystic
fibrosis may not experience problems until reaching his or her teen years – or even adulthood.
For Statistics, Centers for Disease Control and Prevention (CDC), UPMC Children’s Hospital of
Pittsburg and Cystic Fibrosis foundation have all mentioned that approximately, 30,000 people or more have
been diagnosed with Cystic Fibrosis in the United States of America. Worldwide, more than 70,000 people is
listed to have been diagnosed with this disease (Cystic Fibrosis foundation, 2020). This disease is with an
estimated incidence of 1/25,000 to 1/18,000 among Caucasian populations, but is relatively rare in the Asian
populations (Epidemiology and Genetics of Cystic Fibrosis in Asia, 2018). No definitive numbers are listed
regarding the epidemiology of this disease in Asia due to lack of National Registries for this particular disease

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in most countries or simply because the occurrence of this disease among Asians is very unlikely and rare
(Callaghan, et al., 2021).

II. Statement of Objectives

A. General Objectives

This case analysis aims to increase the understanding and knowledge of student nurses on
how to care for patients with Cystic Fibrosis effectively and efficiently.

B. Specific Objectives

Specifically, this case analysis aims to:

1. define Cystic Fibrosis and its effects to the body as a whole;
2. illustrate the pathophysiology of Cystic Fibrosis and in relation to the signs and
symptoms specifically observed in the patient;
3. describe and identify the common signs and symptoms of Cystic Fibrosis;
4. discuss the medical and surgical interventions for the management of Cystic
Fibrosis;
5. formulate appropriate nursing care plans suited for the patient based on the
assessment findings;
6. identify care measures to be given to the patient and family to promote continuity of
care and independence after discharge.

III. Patient’s Profile

Name : Patient X
Ethnic Background : American
Civil Status : Single
Religion : N/A
Occupation : High school student
Admitting Diagnosis : Cystic Fibrosis
Final/Principal Diagnosis: Cystic Fibrosis
Date and Time Admitted : November 22, 2021 – 08:00 AM

IV. Chief Complaint

Was admitted for an extended stay for a probable lung transplant

V. Present History of Illness

The patient has been diagnosed with Cystic Fibrosis at the age of 2. She has been in and out of the
hospital during exacerbations. At the age of 17, her lung capacity’s been reduced to only 35%. Due to impaired
nutrition related to malabsorption, a Gastrostomy tube has been inserted for additional delivery of nutrition. It
later gets infected and the patient had to undergo surgery to replace it.
Her most recent hospital stay was supposed to prepare her for a probable lung transplant. Throughout
her stay, she has been strictly compliant with her treatment regimen and has been observing infection
precautions such as wearing a face mask and wearing latex disposable gloves for added protection from
possible cross-infection. A “six-foot distance” rule has also been observed throughout her stay. Upon arrival of
the viable pair of lungs, the patient had undergone a successful surgery and has recovered very well.

VI. Past History of Illness

Patient X was born terminal via caesarian section with presence of lung complications and her
immunizations were not clearly defined and stated. She suffers from cystic fibrosis, a genetic disease, since 2
years old and began taking treatment, pills, medication and multiple surgeries were also done and performed
including nasal polypectomy, a surgical procedure to remove polyps located in the nasal passages with
gastrostomy tube inserted on her abdomen for additional nutrition to pass through and is in need of lung
transplant. Portable oxygen concentrator was always worn at all times whenever she leaves her room. She
mentioned that she also has obsessive - compulsive disorder that she acquired from her disease.

VII. Family Health History

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 The patient is the second daughter and youngest child of her family. Respiratory illnesses are
hereditary in her family while other diseases such as renal, heart and neurological diseases are not experience
by any of her family members. She mentioned that her older sister died recently from drowning. Her activities
are exerted to a minimum effort such as attending her regimen and personal hygiene, walking, studying and
making YouTube contents.

VIII. Developmental History

The health belief model by Rosenstock is concerned with health-decision making and can be used to
this case study, given that the patient had cystic fibrosis. Perceived susceptibility in the health belief model is
applicable to this condition because the patient believes that she will have a higher chance of worsening of her
cystic fibrosis mostly when cross-infected with others who also suffers from cystic fibrosis and might have
limited lifespan or their lungs would be compromised and won’t be qualified for a transplant. With proper
health teaching and promotion about lifestyle modification with the patient and lessen her control issues but
strict compliance with her regimen will make her feel that this condition can be improved in the future by
using a variety of treatments together with a healthy lifestyle and bring a positive expectation that by taking a
recommended action with this condition will help her to prevent further complications such as risk for sepsis in
this condition as stated by the model.

According to Erick Erickson’s fifth stage of Psychosocial Development which is the ego identity vs.
role confusion, an individual has a strong sense of self wherein he/she upholds his/her values and beliefs
independently which weren’t applied to this patient. In experiences of patient X, social interactions and her
roles as a teenager weren’t achieved much because of the patient’s condition and was confined for a very long
time and can only do things under the nurses’ supervision. Although, she has friends who visits her in the
hospital and has been making Youtube contents wherein she documents her personal exploration and personal
life as an individual who has cystic fibrosis to encourage and educate other people, still, she wasn’t allowed to
do much of what other teenagers supposed to do such as exploring their identities, doing their hobbies in a
different setting and activities including going to adventures, parties, and attending school.

IX. Social and Environmental History

The patient is close to her family especially to her sister before she died as she is to her doctor and
nurses who monitor her. She is self-studying and isolated from everyone as she was diagnosed for a long time
and social interaction is limited because of her condition such as physical contact with other patients with
cystic fibrosis is exempted which is limited to six feet apart only with the risk of cross-infection. She has been
in and out of the hospital and was confined in a private room where she has her own bed, bathroom and space
where her privacy is maintained and where her visitors can visit her and is near to the nurses’ station for close
monitoring. Wearing of face mask and latex gloves, spraying of alcohol and a six feet distance were observed
as infection precaution inside the environment. Food is served at the right time and she also has access to the
kitchen. An emergency button is present in her room whenever complications occur.

X. Lifestyle and Health Practices

Patient X is a 17-year old teenager who had been in and out of the hospital due to her condition. Her
daily meal composed of different variety of foods such as eggs, fresh fruits and vegetables and meat which are
composed of high-calorie and high-fat foods. She goes to the gym for cardio and walking as a form of exercise.
She follows strict compliance with her regimen and spends most of her leisure time studying, going to the
NICU to watch newborn babies and making videos.

XI. Health Assessment

A. General Survey

Patient received fully awake in high-fowler’s position, with intact and dry central line dressing on the
right subclavian vein and gastrostomy tube on the left lower abdomen. Patient is on oxygen inhalation at 2-3
LPM via the nasal cannula.
Patient appears clean and well-groomed wearing a neat clothes, with nails well-trimmed and hair well
kept. No body odors noted. She is able to perform activities of daily living without assistance. Her speech is
clear, moderately paced, and culturally appropriate. Patient establishes good eye contact when conversing with
others. She smiles and frowns appropriately. Patient is cooperative and responsive. She is oriented to the self
and others around her, able to determine the time and date and is aware that she stays in the private room.

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 Patient is slender. She verbalized that she is 5’2” tall and weighs 40 kilograms. Patient’s BMI is 16.21
which is underweight.

B. Head to Toe Assessment

1. Head Normocephalic, hair well distributed, oiliness and flaking noted, no
lice, no areas of pain or tenderness during palpation.
2. Eyes Able to distinguish colors, wears corrective lenses, sclera is anicteric,
pupils are equally round, reactive to light and accommodation, EOM
is intact, able to follow penlight with gaze, no edema or tenderness
over the lacrimal gland and no tearing.
3. Ears Able to understand and hear spoken language correctly, with minimal
cerumen build – up in the ear canal, sliver and intact tympanic
membrane.
4. Nose and Patient is receiving oxygen via the nasal cannula. Nose is obstructed
with nasal flaring noted, septum is located midline, no lesions seen,
Sinuses
patient is unable to distinguish the scent of perfume, sinuses are
inflamed and tender upon palpation, shortness of breath is noted
during daily activities.
5. Mouth Complete set of adult teeth, pearly white in color, and no mal aligned
tooth, had braces for 1 year and a half. No dental caries noted. Oral
mucosa is moist and pinkish, no lesions noted, tonsils are not
inflamed, uvula is located midline.
6. Neck ROM intact, able to change direction of head slowly without
complaints of pain, carotid pulse are bilaterally symmetrical, full and
strong pulses, jugular vein is not distended, superficial cervical lymph
nodes are palpable but non tender. Thyroid is located midline, no
enlargement noted, trachea is located midline.
7. Chest Chest wall is intact with symmetrical chest expansion. Patient is
tachypneic, use of accessory muscles noted, with dyspnea and
occasional chest tightness. Crackles heard upon auscultation on both
lungs, persistent coughing noted with thick sticky mucus production
that often leads to frequent lung infections. Patient with a central line
on the right subclavian vein, dressing is intact and dry. With limited
movement on the right shoulder. Patient maintains high fowlers’
position to facilitate breathing.
8. Cardiac Adynamic pericardium; normal rate, regular rhythm, no murmur
noted, no visible pulsations in the precordium, palpable apical pulse.
9. Breast/Chest Skin color is similar with the rest of the body, nipple is dark colored,
no discharges.
10. Abdomen Rounded, with hyperactive bowels sounds heard in all the quadrants,
soft, no direct tenderness or rebound tenderness upon palpation.
Patient with dry and intact G-tube on the left lower abdomen used for
additional nutrition due to poor growth and poor weight gain in spite
of a good appetite. Patient complains of difficulty in bowel
movements, large, greasy stool noted.
11. Genitals No complaints of dysuria or urinary retention or incontinence. No
inserted catheter.
12. Musculoskeletal Muscle strength is rated as 5/5. No deformities or swelling. No
visible tremors noted. No complaints of pain. Joints move smoothly.
Patient is seen doing moderate exercises.
13. Integumentary Skin is dry and pale with salty taste due to high salt concentration in
sweat, slight clubbing of her fingers is noted. Cuticles are intact and
without inflammations, has a normal capillary refill time of 1 to 2
seconds, and good skin turgor of 1 to 2 seconds. Patient is connected
to a central line catheter on the right subclavian vein and a G-tube on

6
 the left lower abdomen; therefore, small incisions were made,
dressings are dry and intact, no signs of infections noted. Patient is
also on an oxygen inhalation via the nasal cannula.

C. 13 Areas of Assessment
1. Psychosocial and Psychological Status

Patient X is a 17 years old female. She spends most of her time in the hospital as a cystic fibrosis
patient. Patient is a high school student and Youtuber. She uses her YouTube channel to document her life
with CF in order to help educate other people. Patient X’s concerned parents have recently divorced. Both feel
burdened by patient X’s illness and the death of their other daughter. Patient X’s medical expenses and other
financial needs are supported by her parents. Due to illness, her lifestyle is affected. Patients with CF have to
maintain at least 6 feet of distance from each other to avoid dangerous cross-infection, a unique challenge for
her who is falling in love for the first time. The patient and her family have no practices or beliefs which might
affect to providing healthcare.
Based on Erikson’s theory of psychosocial development, the patient falls under ego identity vs. role
confusion since this stage is applicable to ages 12-18 years old. Based on the assessment, patient falls under
this stage for the reason that the patient has been sharing, encouraging and reinforcing through her personal
exploration by documenting her life as an individual who has cystic fibrosis in her YouTube channel to help
educate other people.

2. Mental and Emotional Status

Patient is conscious and coherent, oriented to time, place and person. She can identify things or names
being asked. She is able to understand instructions and acts according to her age and maintain eye contact. She
can recall recent and remote memories she experienced. She is responsive and collaborative to verbal
commands, touch and painful stimuli.

3. Environmental Status

Ms. X is in the special isolation room. There are no sensory deficits. All her things are organized and
she is aware that she is in the hospital. Patient’s environment is clean, there is steady pattern of activity, light
noise and color in her environment and it does not distract her. The food and water are at the side table and is
placed at the right side of the patient, it is accessible for her.

4. Sensor Status
a. Visual Status

Patient’s irises are black in color and sclera is whitish in color, eyebrows and eyelashes are
evenly distributed. The patient is using corrective eyeglasses when reading and pupils are equally round
and reactive to light and accommodation. Her eyes can follow the six cardinal positions and eyes were
able to move in full range of motion in all directions.

b. Auditory

Patient’s ears are symmetrical with minimal cerumen build-up observed. No lesions and
tenderness upon palpations. No corrective auditory deficits and no auditory device being used by the
patient. Patient was able to repeat the whispered words on both ears when whisper test was conducted.

c. Olfactory Status

Patient’s nose has no deviation in terms of shape and size. There are discharges seen during
the assessment. Orange peel and apple peel were used for this test and the patient was not able to
differentiate both smells from each other signifying that there are obstructions. She has decreased sense
of smell and shortness of breath during activity noted.

d. Gustatory Status

Patient is not using dentures. She has slightly dry lips. According to her, she has no difficulty
in masticating and swallowing and has good appetite. She has intact gag reflex.

e. Tactile Status

She can distinguish light from firm touch on her body and is able to perceive hot and cold

7
 sensations in proportion to stimulus and there are no aberrant sensations.

5. Motor Status

Motor strength was assessed and is rated as 5/5. Her movement is limited since she has right
subclavian central line and gets tired easily. The patient can move all her joints smoothly. No prosthetics
device was noted present in the patient and all her extremities are intact. Peripheral pulses were present such as
radial. Extremities are warm to touch.

6. Thermoregulatory Status

The normal temperature can range from 36. C to 37.5 C for a healthy adolescent. The patient
temperature level indicate that she has no fever.

Date Time Temperature

November 22, 2021 (admission) 8am 37.2 °C
10am 36.9 °C
2pm 36.7 °C
8am 37 °C
November 23, 2021 10am 36.6 °C
2pm 37.2 °C
8 am 36.8 °C
November 24, 2021 10 am 37 °C
2 pm 36.7 °C

7. Respiratory Status

The normal respiratory rate for adolescent is 12-20bpm and SPO2 of 95%-100%. The RR of the
patient was high ranging from 23-26 while her SPO2 is low during the admission and for the entire
hospitalization due to loss of lung function.

Date Time RR SPO2

8am 26 bpm 63 %
November 22, 2021
10am 24 bpm 65 %
(admission)
2pm 23 bpm 68 %
8am 25 bpm 68 %
November 23, 2021 10am 24 bpm 64 %
2pm 23 bpm 72 %
8am 24 bpm 70 %
November 24, 2021 10am 23 bpm 68 %
2pm 25 bpm 68 %

8. Circulatory Status

A normal cardiac rate of an adolescent is 60-100 bpm, normal BP is 120/80 and normal capillary refill
is 1-2 seconds. Patient X’s cardiac rate and capillary refill are within normal range, however, her blood
pressure is low upon admission but was within the normal range the following days.

Date Time CR BP Capillary Refill

8am 99 bpm 90/60 mmHg 1-2 seconds
November 22, 2021
10am 97 bpm 100/70 mmHg 1-2 seconds
(admission)
2pm 95 bpm 100/80 mmHg 1-2 seconds
8am 96 bpm 90/80 mmHg 1-2 seconds
November 23, 2021 10am 98 bpm 110/70 mmHg 1-2 seconds
2pm 96 bpm 120/80 mmHg 1-2 seconds
8am 95 bpm 110/80mmHg 1-2 seconds
November 24, 2021 10am 94 bpm 120/80 mmHg 1-2 seconds
2pm 98 bpm 120/80 mmHg 1-2 seconds

9. Nutritional Status

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 The patient’s food is being served in the hospital and she is in a balance diet consisting of fat, protein,
fruits and vegetables. The patient has also G-tube located in her left lower abdomen to directly deliver
additional nutrition. The patient’s appetite is good. There is no culture or religious dietary restrictions reported
by the patient.

10. Elimination Status

The patient eliminates in a toilet bowl two times a day. The stool is usually large and greasy. She
sometimes complains of constipation. She drinks water to aid elimination. She verbalized that she regularly
urinates during her stay at the hospital. She usually consumes 5-6 glasses of water per day. She claimed
absence of other special problem like urinary and bowel retention and urinary incontinence.

11. Sleep, Rest and Comfort Status

She claimed that she is comfortable with her sleep even if she is in the special isolation room but
sometimes being disturbed especially at night when nurses have to check on her vital signs or give her
medications.

12. Fluids and Electrolytes Status

The patient usually drinks 5-6 glasses of water daily and urinates regularly. The patient denies the
feeling of thirst. Her skin turgor is normal and she has moist mouth and mucus membranes. The patient’s
capillary refill is 1-2 seconds.

13. Integumentary Status

Patient’s skin color is pale and has a good skin turgor, no skin allergy and no bed sore or edema noted
upon inspection. With central line inserted in the right subclavian and G-tube sited on her left lower abdomen.
She wears a fairly neat clothes and nails are trimmed short with slightly clubbing around the toenails and
fingernails. Her hair is brown and well-kept.

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XII. Diagnostics

Significance/ Purpose of the Date of

Diagnostic Procedure Description of Procedure Findings & Implications
Procedure Procedure

Chest X-Ray An X-ray is an imaging test that uses small amounts of Chest X-ray test can detect problems November 22, Findings:
radiation to produce pictures of the organs, tissues, and that includes cough, shortness of breath, 2021 The result reveals hyperinflation, bronchial
bones of the body. When focused on the chest, it can help chest pain, poor oxygenation (hypoxia), thickening and dilatation, peribronchial cuffing,
spot abnormalities or diseases of the airways, blood back pain, chest injury, and fever. This cystic radiolucencies, an increase in interstitial
vessels, bones, heart, and lungs. Chest X-rays can also procedure can help them diagnose, markings, and scattered nodular densities.
determine if there are fluids in the lungs, or air monitor, and treat many medical
surrounding the lungs. conditions. Implications:
Cystic fibrosis affects more than the lungs. It affects
A chest X-ray is an easy, quick, and effective test that has A chest X-ray is often among the first the reproductive system, the sinuses, the kidneys, the
been useful for decades to help doctors view some of your procedures the doctor suspects heart or liver, the digestive system, and overall bone density
most vital organs. lung disease. Chest X-rays is used to that is why chest x-ray is one of the diagnostic test to
further know how well certain be performed in order to monitor if there are present
REFERENCE/S: treatments are working and to check for injuries and other condition that may trigger
Krans, B. (2012, August 7). Chest X-Ray. Healthline; complications after certain procedures complications.
Healthline Media. or surgeries.
https://www.healthline.com/health/chest-x-ray

Sweat (Chloride) Test The sweat test measures the amount of chloride (a The sweat test is a reliable test for the November 22, Findings:
component of salt) in the sweat. There are no needles diagnosis of Cystic Fibrosis in 2021 A cystic fibrosis patient reveals a sweat chloride test
involved in this test. Sweat testing is painless and lasts approximately 98% of patients with result of less than 30 mmol/L.
less than an hour. Cystic Fibrosis. It's also called an
iontophoretic sweat test or chloride Result ranges:
The test is performed by placing a solution on the forearm sweat test. It's used primarily for people  Less than or equal to 29 mmol/L = CF is unlikely
(or the thigh if the child is too small) and attaching who have symptoms of cystic fibrosis regardless of age*.
electrodes. The skin is stimulated to sweat with a mild (CF). The body's natural chemistry  Between 30 - 59 mmol/L = CF is possible and
electric current, which does not cause pain or harm your requires the right balance of sodium and additional testing is needed.
child. The sweat is collected onto a gauze pad and chloride.  Greater than or equal to 60 mmol/L = CF is
analyzed. Higher than normal amounts of chloride may

10
 suggest cystic fibrosis. likely.

REFERENCE/S: Implication:
Sweat Test. (2021). Cystic Fibrosis Foundation. As Cystic Fibrosis is caused by a faulty gene that
https://www.cff.org/intro-cf/sweat-test controls the movement of chloride and water into and
out of cells, people with cystic fibrosis often sweat
‌ more than people without the condition, and this
sweat contains high levels of chloride, which can
crystallise into salt visibly on the skin. Thus, sweat
test could actually help in the diagnosis of cystic
fibrosis for both adult and children.

Complete Blood Count This routine blood test or CBC measures the cells in the It was performed to the patient to November 22, Findings:
body through the blood. They test the blood for white determine general health status, screen, 2021 The test reveals that CFTR (cystic fibrosis
blood cells, red blood cells, hemoglobin, hematocrit, and diagnose, or monitor any one of a conductance transmembrane regulator) causes
platelets. CBC tests can detect if a person has anemia, variety of diseases and conditions that changes in the body's cell's electrolyte transport
nutritional deficiencies, an infection in the respiratory affect blood cells, infection, system which explains that electrolytes are substances
system, cancer, and bone marrow problems. inflammation, bleeding disorder, or in blood that are critical to cell function.
cancer.
The sample is most commonly obtained from a vein Implications:
visible from the skin, such as a vein on the back of the According to a Research conducted by the Georgia
hand or the inner angle of the elbow (antecubital fossa). Health Sciences University, 2012, Cystic Fibrosis can
affect blood vessel dysfunction due to the reduced
This sample is then taken to the laboratory for analysis, ability of blood vessels to respond to important cues,
and the complete blood count results may be available such as dilating when exercise or stress increase the
within hours after collection. body's demand for blood and oxygen.

REFERENCE/S:
Complete Blood Count: Normal Ranges & Test Details.
(2021). Cleveland Clinic.
https://my.clevelandclinic.org/health/diagnostics/40
53-complete-blood-count

Pulmonary Function These tests help measure the lungs' ability to exchange These tests were performed to November 23, Findings:
Tests oxygen and carbon dioxide correctly. They are often done determine how well the lungs receive, 2021 The test reveals that there was loss of pulmonary
with special machines that a child or adult must breathe hold, and utilize air. To monitor a lung function, indicating progressive lung disease as

11
 into. It is non-invasive test that show how well the lungs disease. Thus, to monitor the evidenced by peripheral airway obstruction, with
are working. The tests measure lung volume, capacity, effectiveness of treatment and to retention of secretions and inflammation result in a
rates of flow, and gas exchange. determine the severity of a lung disease. cycle of tissue damage that is accompanied by
bronchiectasis.
A patient sit in a clear airtight box that looks like a phone
booth. The technologist will instruct the patient to breathe Implications:
in and out of a mouthpiece. Changes in pressure inside the The uses of pulmonary tests are useful in assessing
box help determine the lung volume. Lung volume can lung disease severity and progression. Lung disease
also be measured when you breathe nitrogen or helium in Cystic Fibrosis patients is essentially obstructive.
gas through a tube for a certain period of time. Spirometry test, and volume-time and flow-volume
curves are the most common pulmonary tests
REFERENCE/S: performed to help in the diagnosing and management
Stanford Health Care. (2017, September). of cystic fibrosis.
Stanfordhealthcare.org.
https://stanfordhealthcare.org/medical-conditions/c
hest-lungs-and-airways/adult-cystic-fibrosis/
diagnosis/pulmonary-function-tests.html?
fbclid=IwAR1q4_ua5YVtHSi9LPDAPkTQqoqzW
BQQUif7GWO_UX6Oia4O99qSAxLlU4I#:~:text
=Spirometry%20%2D%20A%20spirometer%20is
%20a,receive%2C%20hold%2C%20and%20utilize
%20air

Sputum Culture Test This test is done on the material that is coughed up from A sputum (mucus) CF respiratory November 23, Findings:
the lungs and into the mouth. A sputum culture is often screen or culture help the doctors detect 2021 The test reveals dark brown, tenacious phlegm. The
done to find out if an infection is present. Sputum, also and identify these bacteria or fungi so phlegm is brown because of blood and the intense
known as phlegm, is a thick type of mucus made by the they can prescribe the most effective chronic inflammation that comes with the chronic
lungs. antibiotics to target a specific infection. disease state. Cystic Fibrosis (CF) diagnostic
A sputum culture can help identify microbiology has evolved from a focus on
The patient will be asked to cough deeply and spit any specific causes of infections in the lungs Staphylococcus aureus as primary pathogen to
phlegm that comes up from the lungs into a special and airways. Such infections can lead to identification of the contribution of Pseudomonas
container. The sample is sent to a lab. The sample is then coughing that produces yellow, aeruginosa and other non-fermenting gram negatives
placed on a special plate that enables growth of certain greenish, or blood-tinged sputum, in
bacteria and fungi if an infection is present. addition to fever and difficulty Implications:
breathing. Tiny hairs on the outside of cells, called cilia, propel
REFERENCE/S: the mucus out of the lungs and into the throat where

12
Burns, J. L., & Rolain, J.-M. (2014). Culture-based the mucus can be swallowed or coughed out. In
diagnostic microbiology in cystic fibrosis: Can we people with cystic fibrosis, mucus is dehydrated,
simplify the complexity? Journal of Cystic becoming so thick and sticky that the cilia are unable
Fibrosis, 13(1), 1–9. to propel mucus out of the lungs. Sputum Test was
https://doi.org/10.1016/j.jcf.2013.09.004 performed because this will determine if the patient
‌ has another cause of infection and to determine if the
condition is becoming worse.

13
 XIII. Comprehensive Pathophysiology

PREDISPOSING FACTOR PRECIPITATING FACTOR

Genetics, family history, race Exposure to crowds or people with

known infections (acquisition of bacteria
Mutation of the CFTR gene on chromosome 7
like P. aeruginosa, B. cepacia, and S.
Defective CFTR protein = Defective CFTR channel aureus), air pollution

Impaired reabsorption of Cl- and Na+ Impaired ion and H2O transport (Na+Cl-)

Increased secretion of Cl- and Na+ In duct epithelial tissue of the other parts of the body,
into sweat glands CFTR proteins facilitate diffusions of Cl- into secretions

Impaired Cl- diffusion into ciliary fluids Goblet cells proliferation

Salty sweats
Impairment of the airway surface liquid Increased mucus production
(ASL) of the conducting airways

Decreased clearance of mucociliary secretions

Pancreas Accumulation of secretions in the secretory passages throughout the body

Pathogens gets trapped in mucociliary secretions and proliferate

Lung Tissue Inflammation

Neutrophils migrate into the alveoli in

response to inflammatory stimulus
Pulmonary capillaries
do not adequately Pulmonary edema
Neutrophil infiltration and
proinflammatory cytokines absorb fluid
lead to tissue edema,
dysfunction, and subsequent Then body’s attempt to
destruction of pulmonary heal lung tissue result in Impaired Gas Exchange
epithelium deposition of hyaline
membranes in the alveoli
CD4 helper T-cells activated
to stimulate B-cells

Clearance of pathogen by
Persistent bacteria in airways
adaptive immune response

Structural damage Epithelial obstruction

Tissue damage of airways
to bronchial walls
Risk for Further impairment
Bronchospasm
Aspirations of bacterial clearance

Persistent inspiratory
adventitious breath sounds
(crackles > wheezing)

Ineffective Airway Clearance

14
XIV. Treatment/Management
A. Drugs

INDICATION /
DRUG NAME MECHANISM OF ACTION ADVERSE EFFECT NURSING RESPONSIBILITIES
CONTRAINDICATION
GENERIC: Albuterol attaches to beta2 receptors on INDICATION/s: CNS: BEFORE:
ALBUTEROL SULFATE bronchial cell membranes, which  Relief and prevention of anxiety, dizziness, drowsiness, a. Checked doctor’s order.
stimulates the intracellular enzyme bronchospasm in patients with headache, hyperkinesia, insomnia, b. Assessed vital signs.
BRAND: adenylate cyclase to convert adenosine reversible obstructive airway irritability, nervousness, tremor, c. Assessed for any allergies.
AccuNeb triphosphate (ATP) to cyclic adenosine disease. vertigo, weakness d. Assessed possibility for
monophosphate (cAMP). This reaction  Treatment of acute attacks of interactions with other drugs the
CLASS: decreases intracellular calcium levels. It bronchospasm. CV: patient may taking.
also increases intracellular levels of  Prevention of exercise-induced angina; arrhythmias, including atrial
THERAPEUTIC: cAMP, as shown. Together, these effects bronchospasm. fibrillation, extra DURING:
Bronchodilators relax bronchial smooth-muscle cells and systoles, supraventricular a. Explained the purpose and
inhibit histamine release. CONTRAINDICATION/s: tachycardia, and tachycardia; chest importance of the drug to the
PHARMACOLOGIC: Hypersensitivity to albuterol or its pain; hypertension; hypotension; patient or SO.
Adrenergics REFERENCE/S: components. palpitations b. Educated patient how to use
(2021, August 13). Pediatriconcall.com. inhaler.
DOSAGE: https://www.pediatriconcall.com/dr DRUG TO DRUG EENT: c. Instructed patient to shake
ugs/albuterol/234 altered taste, dry mouth and throat, canister before use and to check
2 inhalations every 4 to 6 INTERACTION/s:
hours Antiarrhythmics (amiodarone, ear pain, glossitis, hoarseness, that a new canister is working by
bretylium, disopyramide, dofetilide, oropharyngeal edema, pharyngitis, spraying it the appropriate
ROUTE: procainamide, quinidine, sotalol), rhinitis, taste perversion number of times (once to four
Inhalation arsenic trioxide, chlorpromazine, times based on manufacturer
dolasetron, droperidol, mefloquine, ENDO: instructions) into the air while
mesoridazine, moxifloxacin, hyperglycemia looking for a fine mist.
pentamidine, pimozide, tacrolimus,
thioridazine, ziprasidone: May GI: AFTER:
prolong QT interval and increase risk anorexia, diarrhea, dysphagia, a. Monitored for effectiveness.

15
of life-threatening arrhythmias, heartburn, nausea, vomiting b. Monitored patient compliance to
including torsades de pointes. drug therapy.
Monitor QT interval and patient. GU: c. Monitored for side effects and
CNS stimulants: May increase CNS UTI adverse drug reactions.
stimulation. Avoid using together. d. Warned patient not to exceed
Digoxin: May decrease digoxin level. MS: prescribed dose or frequency. If
Monitor digoxin level closely. muscle cramps doses become less effective, tell
Diuretics (furosemide, thiazides): patient to contact his prescriber.
May cause ECG changes and RESP: e. Instructed patient to immediately
hypokalemia. Monitor potassium bronchospasm, cough, dyspnea, report signs and symptoms of
level. Use caution when administered paradoxical bronchospasm, allergic reaction, such as
with non-potassium-sparing diuretics. pulmonary edema difficulty swallowing, itching,
Linezolid, MAO inhibitors, TCAs: and rash.
May increase adverse CV effects. SKIN:
Consider alternative therapy. Monitor diaphoresis, flushing, pallor,
patient closely if used together. pruritus, rash, urticaria
Propanolol and other beta blockers:
May cause mutual antagonism. Other:
Monitor patient carefully. angioedema, hypokalemia,
Theophyllines: May decrease infection, metabolic acidosis
theophylline plasma concentration.
Adjust dosage as needed and monitor
patient.

DRUG TO FOOD
INTERACTION:
______________

16
GENERIC: Dornase alfa cleaves the DNA released INDICATION/s: GENERAL: BEFORE:
DORNASE ALFA from the neutrophils and reduces mucous It is indicated for daily administration Voice alteration, a. Checked and verified with
viscosity and further prevent airway in conjunction with standard Sore throat, doctor’s order and Kardex.
BRAND: infections and damage to the lung therapies for the management of Rash, b. Assessed vital signs.
Pulmozyme parenchyma. Overall, improving cystic fibrosis patients to improve Laryngitis, c. Assessed for any allergies.
pulmonary function. pulmonary function. Eye redness, d. Observed rights in medication
CLASS: Irritation of the eye, administration such as giving the
Mucolytics REFERENCE/S: CONTRAINDICATION/s: Inflammation of the eye, right drug to the right patient
Pulmozyme. (2021, August 25). RxList; Contraindicated in patients with Runny or stuffy nose, using the right route and at the
DOSAGE: RxList. known hypersensitivity to dornase Hives, right time.
2.5 mg single-use ampule once https://www.rxlist.com/pulmozyme- alfa. Difficulty breathing,
drug.htm Swelling of face, lips, tongue or
daily
ROUTE: throat,
‌
Inhalation DRUG TO DRUG Chest pain, DURING:
INTERACTION/s: Fever a. Explained the purpose and
______________ importance of the drug to the
patient or SO.
DRUG TO FOOD b. Avoided combining other drugs
INTERACTION: in the nebulizer to avoid the
______________ formation of precipitates and
potential loss of effectiveness of
either drug.
c. Cautioned patient to store the
drug in the refrigerator,

17
 protected from light.
d. Provided thorough teaching,
including the drug name and
prescribed dosage, measure to
help avoid adverse effects,
warning signs that may indicate
problems and the need for
periodic monitoring and
evaluation to enhance patient
knowledge about drug therapy
and to promote compliance.

AFTER:
a. Monitored for adverse effects.
b. Advised the patient to avoid
driving or performing dangerous
tasks if dizziness and drowsiness
occur to prevent patient injury.
c. Monitored patient’s response to
the drug.

GENERIC: Piperacillin is an extended-spectrum INDICATION/s: CNS: BEFORE:

PIPERACILLIN SODIUM penicillin antibiotic, but it can be It is indicated for the treatment of headache, insomnia, agitation, a. Checked the doctor’s order.
AND TAZOBACTAM SODIUM destroyed by an enzyme produced by patients with moderate to severe dizziness, anxiety, fever, pain b. Verified patient’s identity.
bacteria called beta lactamase. Tazobactam infections caused by susceptible c. Obtained history before
BRAND: inhibits beta lactamase and prevents the isolates of the designated bacteria in CV: hypertension, tachycardia, initiating therapy to determine
Zosyn destruction or piperacillin. Therefore, the conditions such as intra- chest pain, edema previous use of and reactions to
tazobactam is given with piperacillin to abdominal infections, skin and skin penicillins or cephalosporins.
CLASS: enhance the activity of piperacillin in structure infections, female pelvic EENT: Persons with a negative history
eradicating bacterial infections. infections and pneumonia. rhinitis of penicillin sensitivity may still

18
THERAPEUTIC: Piperacillin kills bacteria by inhibiting the GI: have an allergic response
Antibiotic synthesis of bacterial cell walls. It binds CONTRAINDICATION/s: diarrhea, nausea, constipation, d. Obtained specimens for culture
preferentially to specific penicillin-binding Contraindicated in patients with vomiting, dyspepsia, stool changes, and sensitivity prior to initiating
PHARMACOLOGIC: proteins (PBSs) located inside bacterial history of allergic reactions to any of abdominal pain therapy.
Extended-spectrum cell walls. PBPs vary among bacterial the penicillins, cephalosporins, or
penicillin/beta-lactamase species, and thus susceptibility to beta-lactamase inhibitors. GU: DURING:
inhibitor piperacillin depends on the ability of interstitial nephritis. a. Assessed patient for infection
piperacillin to bind to each species’ DRUG TO DRUG Hematologic: leukopenia, anemia, (vital signs; appearance of
DOSAGE: specific PBPs. INTERACTION/s: eosinophilia, thrombocytopenia wound, sputum, urine and stool;
3.375g every 6 hours (12.0g The combination of piperacillin and Aminoglycosides: Substantially WBC) before and during the
piperacillin/1.5g tazobactam in a tazobactam has activity against a variety of inactivates aminoglycoside. Don’t Respiratory: therapy
day) gram-positive and gram negative aerobic mix in same I.V. container. dyspnea b. Administered by infusion over a
and anaerobic bacteria. Heparin, oral anticoagulants, other period of atleast 30minutes.
ROUTE: ‌ drugs affecting blood coagulation Skin: c. Observed for signs and
IV REFERENCE/S: system or thrombocyte rash (including maculopapular, symptoms of anaphylaxis. Kept
Omudhome Ogbru, PharmD. (2019, function:  Prolongs bullous, urticarial, and eczematoid), epinephrine, an antihistamine,
October 15). piperacillin and effectiveness. Monitor coagulation pruritus, inflammation and phlebitis and resuscitation equipment
tazobactam (Zosyn). MedicineNet; parameters frequently. close by in the event of an
at I.V. site
MedicineNet.
Probenecid: Increases blood levels anaphylactic reaction.
https://www.medicinenet.com/piper
acillintazobactam_sodium- of piperacillin/ Other:
injection/article.htm tazobactam. Probenecid may be used candidiasis, anaphylaxis AFTER:
for this purpose. a. Monitored patient with mild to
Vecuronium:  Prolongs moderate rash progression. If
neuromuscular blockade of rash becomes severe,
vecuronium. Monitor patient closely. discontinue immediately.
b. Cautioned patient to notify the
DRUG TO FOOD health care professional if fever
INTERACTION: and diarrhea occur, especially if
______________ stool contains blood, pus, or

19
 mucus.
c. Advised patient not to treat
diarrhea without consulting
health care professional.

GENERIC: Tobramycin is bactericidal; it binds INDICATION/s: CNS:  BEFORE:

TOBRAMYCIN SOLUTION directly to the 30S ribosomal subunit, It is indicated for serious infections fever (ophthalmic administration); a. Checked the doctor’s order.
FOR INHALATION thereby inhibiting bacterial protein caused by Escherichia coli, headache, lethargy, b. Verified patient’s identity.
synthesis. Its spectrum of activity includes Staphylococcus aureus, and Proteus, confusion, seizures,  disorientation c. Checked for allergies.
BRAND: many aerobic gram-negative organisms, Klebsiella, Enterobacter, Serratia, (injectable form) d. Assessed baseline status.
TOBI including most strains of P. aeruginosa and Pseudomonas, Citrobacter, and e. Don’t premix tobramycin with
some aerobic gram-positive organisms. Providencia species. other drugs; administer
CLASS: Tobramycin may act against some EENT:  separately at least 1 hour apart.
bacterial strains resistant to other CONTRAINDICATION/s: ototoxicity (injectable form); blurred
THERAPEUTIC: aminoglycosides; many strains resistant to Contraindicated in patients vision (ophthalmic ointment); DURING:
Antibiotic tobramycin are susceptible to amikacin, hypersensitive to drug or other burning or stinging on instillation, a. Assessed patient for signs of
gentamicin, or netilmicin. aminoglycosides. Use injectable form lid itching or swelling, conjunctival infection (vital signs; appearance
cautiously in elderly patients and erythema (ophthalmic of wound, sputum, urine and
PHARMACOLOGIC:
administration) stool; WBC) before and during
Aminoglycosides REFERENCE/S: those with impaired renal function or
the therapy
tobramycin. (2021). Glowm.com. neuromuscular disorders. b. Instructed patient on proper
DOSAGE: https://glowm.com/resources/glowm administration (inhalation) and
/cd/pages/drugs/t037.html#:~:text=I DRUG TO DRUG how to use inhalers.
Single-use 5-ml (300-mg)
nhalation%20form%20of
ampule INTERACTION/s: GI: 
%20tobramycin%20is,aeruginosa
%20infection.&text=Monitor Aminoglycosides, amphotericin B, vomiting, nausea, diarrhea AFTER:
ROUTE: %20patient%20for%20symptoms capreomycin, cephalosporins, (injectable form) a. Monitored patient for symptoms
Inhalation %20of%20toxicity.&text=Monitor cisplatin, methoxyflurane, of toxicity.
%20peak%20and%20trough polymyxin B, vancomycin: Increases GU:  b. Advised patient that inhalation
%20drug,be%20below risk of nephrotoxicity, ototoxicity, increased urinary excretion of doses should be taken as close to
%202%20mcg%2Fml. and neurotoxicity. Monitor patient casts; nephrotoxicity (injectable 12 hours apart as possible and
no less than 6 hours apart.

20
 carefully. form).
Antiemetics, antivertigo drugs,
dimenhydrinate: May mask Hematologic: 
tobramycin-induced ototoxicity. anemia, eosinophilia, leukopenia,
Monitor patient closely. thrombocytopenia,
Bumetanide, ethacrynic acid, granulocytopenia (injectable form)
furosemide, mannitol, urea:
Increases risk of ototoxicity. Monitor Respiratory: 
patient closely. bronchospasm (inhaled form)
General anesthetics, neuromuscular
blockers, succinylcholine, Skin: 
tubocurarine: Potentiates rash, urticaria, pruritus (injectable
neuromuscular blockade. Monitor form)
patient closely.
Penicillins: Physically and Other: 
chemically incompatible. Don’t mix hypersensitivity
in same I.V. line. reactions, overgrowth of
nonsusceptible organisms
DRUG TO FOOD (ophthalmic administration)
INTERACTION:
______________

GENERIC: Both Elaxacaftor and Tezacaftor work as INDICATION/s: GENERAL: BEFORE:

LEXACAFTOR, correctors. They bind to the faulty CFTR Trikafta is indicated for the treatment Headache, a. Checked doctor’s order.
TEZACAFTOR, IVACAFTOR protein and help it fold correctly. This of cystic fibrosis in patients aged 12 Upper respiratory tract infection, b. Assessed vital signs.
TABLETS way, cells can shuttle more of it to the years and older. Abdominal pain, c. Assessed for any allergies.
membrane instead of degrading it. Diarrhea, d. Assessed possibility for
BRAND: Ivacaftor-also marketed as Kalydeco-is a CONTRAINDICATION/s: Rash, interactions with other drugs the
Trikafta potentiator: it binds to the CFTR protein Hypersensitivity to Elexacaftor, Runny or stuffy nose, patient may taking.

21
 and holds the channel open so that more Eezacaftor and Ivacaftor. Sneezing or post-nasal drip,
CLASS: salt can pass through it. With a Influenza, DURING:
Cystic Fibrosis transmembrane combination of all three therapies, more DRUG TO DRUG Sinusitis, a. Explained the purpose and
regulator (CFTR) modulator CFTR protein reaches the cell membrane INTERACTION/s: Increased blood bilirubin, importance of the drug to the
and these proteins are more active and ______________ Increased blood creatinine patient or SO.
DOSAGE: functional. phosphokinase b. Combined Trikafta with food
2 tablets (Elexacaftor 100mg, DRUG TO FOOD that contains fat such as butter,
Tezacaftor 50mg, Ivacaftor 75 mg) REFERENCE/S: INTERACTION: peanut butter, eggs, nuts, meat,
in the morning Trikafta. (2021, October 14). RxList; ______________ whole milk, cheese or yogurt.
RxList. c. Instructed the patient to swallow
1 Ivacaftor 150mg in the https://www.rxlist.com/trikafta- the Trikafta tablet whole, do not
drug.htm
evening approximately 12 hours crush chew or break it.
apart d. Instructed the patient not to
change the dose or dosing
ROUTE: schedule without the doctor’s
Oral advice.
e. Educated patient to avoid taking
grapefruit products because it
may lead to unwanted side
effects.

AFTER:
a. Monitored for adverse drug
reactions.
b. Advised the patient to avoid
driving or performing dangerous
tasks until knowing how the
Trikafta will affect.
c. Instructed patient to immediately
report if there are signs of

22
allergic reaction such as hives,
difficulty breathing, and
swelling of face, lips, tongue or
throat.

23
XV. Nursing Care Plans

A. Prioritization of Problems
a.1. List of Problems
1. Ineffective airway clearance related to retained secretions

2. Impaired Gas Exchange related to decreased surface area for gas exchange and
impaired lung function

3. Risk for Aspiration related to the presence of secretions and spasms

4. Imbalanced Nutrition: Less than body requirements related to chronic

malabsorption

5. Risk for infection related to damage to the body's defenses and presence of mucus as
a medium for growth of the organism

a.2. Basis for Prioritization

NURSING DIAGNOSES JUSTIFICATION

Ineffective Airway Clearance is the number 1 out of 5

problems because airway must be given the first attention
1. Ineffective airway clearance based from the rule of ABC (Airway, Breathing and
related to retained secretions Circulation). Also, difficulty of breathing can cause anxiety to
the patient that is why, immediate attention must be done.
Addressing this problem to a proper health care provider will
give patent airway to the patient.

2. Impaired Gas Exchange Based on Maslow's hierarchy of needs, this problem is also
related to decreased surface under the first level, physiological needs; however, in the ABC
area for gas exchange and loss system, impaired gas exchange is included in breathing
of lung function problems. Airways always come first before breathing.

3. Risk for Aspiration related to Risk for Aspiration is the number 3 out of 5 problems because
the presence of secretions and it is still related to airway and risk is what will likely to happen
bronchospasm in the future if interventions were not met properly.

4. Imbalanced Nutrition: Less Based on Maslow's hierarchy of needs, this problem is also
than body requirements under the first level, physiological needs, because food is
related to chronic one of the basic needs of human but, unlike with number 1,
malabsorption 2 and 3 this is not included in ABC system therefore, it
comes after them.

5. Risk for infection related to

damage to the body's defenses This problem places last because it is a potential risk and is
and presence of mucus as a considered the 5th priority after problem number 4 because it is
medium for growth of the under the second level of Maslow's Hierarchy of needs, safety
organism needs.

24
 B. Nursing Care Plans

NCP 1: Ineffective Airway Clearance related to retained secretions secondary to cystic fibrosis
ASSESSMENT EXPLANATION OF THE OBJECTIVES NURSING INTERVENTIONS RATIONALE EVALUATION
PROBLEM

SUBJECTIVE: As shown in the STO: Dx: Dx: STO:

“Cystic fibrosis, as pathophysiology, an ineffective Within 30 minutes-1 hour of ● Assessed cough for ● An effective mucus- (Goal Met)
you know is a genetic cough compromises airway nursing intervention, patient will effectiveness. clearing is important for Within 30 minutes-1 hour
disease that basically clearance and prevents mucus be able to: adequate airway clearance. of nursing intervention, the
makes my body from being expelled, results in a. understand the ● This is to detect patient understood the
produce a sh** ton of airway inflammation which can importance of chest ● Auscultated breath sounds. adventitious breath importance of chest
mucus,” as stated by lead to productive cough with physiotherapy; and sounds. physiotherapy and was able
the patient. thick yellow sputum and results b. expectorate at least a ● Observed for breathing ● Unusual breathing patterns to
to ineffective airway clearance. spoonful of sputum patterns. may imply an underlying expectorate at least a
every hour disease process or spoonful of sputum every
OBJECTIVE: REFERENCE/S: dysfunction. hour.
● Productive cough Ineffective Airway Clearance – ● Unequal chest expansion
● With oxygen per Nursing Diagnosis & LTO: ● Assessed the chest wall for can indicate a LTO:
nasal cannula Care Plan. (2016, August After 72 hours of nursing pneumothorax– a (Goal Met)
even chest expansion.
22). Nurseslabs. interventions, the patient will: complication of cystic
● Labored breathing After 72 hours of nursing
https://nurseslabs.com/ine
● Chest pain a. be proficient in using fibrosis. intervention, patient was
ffective-airway-clearance/
● Crackles heard effective airway ● A decreasing amount and proficient in using effective
upon auscultation clearance therapies to frequency of sputum airway clearance therapies
● Assessed sputum for color,
● Purulent clear secretions on a production, lighter color, to clear secretions on a
amount, and consistency.
secretions ‌ daily basis, such as and thinner consistency of daily basis such as
coughing exercises; sputum indicate coughing exercises,
NURSING b. participate and show improvement in participated and showed
DIAGNOSIS: compliance to exacerbation. compliance to

25
 recommended ● Hypoxemia may result recommended therapeutic
Ineffective Airway therapeutic regimen; ● Monitored oxygen saturation from impaired gas regimen and has improved
Clearance related to and and arterial blood gas levels. exchange and from the arterial blood gas markers.
retained secretions c. show improved arterial buildup of secretions and
secondary to cystic blood gas markers bronchial constriction.
fibrosis
Tx:
Tx: ● To facilitate adequate
● Maintained a clear airway by clearance of secretion.
encouraging patient to
mobilize own secretions with
successful coughing. ● A tripod position permits
● Placed patient in a tripod maximum lung excursion
position for maximum lung and chest expansion.
expansion ● To help patient breathe
● Performed chest more freely and to get
physiotherapy as indicated. more oxygen into the body
● Bronchodilators are
● Administered bronchodilators administered to treat
before ACT as ordered. hyperactivity and to
reverse bronchospasm.

Edx:
Edx: ● Extra activity can worsen
● Encouraged frequent rest shortness of breath.
periods and teaches patient to
pace activity. ● This allows sufficient
mobilization of secretions.
● Educated patient and

26
 significant others on proper
breathing, coughing, and ● Habitual cough
splinting methods. suppression results in the
● Encouraged a frequent and retention of mucus; patient
effective cough- particularly should be encouraged to
around airway clearance actively and intentionally
therapy (ACT). use effective coughing to
clear airway mucus.
● Allows the patient to be
compliant of therapy.

● Explained the importance of ● To show patient the

compliance with chest abnormal signs to take
physiotherapy. note.
● Discussed normal vs ● To allow patient perform
abnormal ABG markers. proper coughing exercises.

● Instructed patient on how to

perform proper coughing
exercises.

27
NCP 2: Impaired Gas Exchange related to decrease surface area for gas exchange and loss of lung function as evidenced by crackles on both lungs and shortness of breath
ASSESSMENT EXPLANATION OF THE OBJECTIVES NURSING INTERVENTIONS RATIONALE EVALUATION
PROBLEM

SUBJECTIVE In the pathophysiology, impaired STO: Dx: Dx: STO:

“Like all kids with gas exchange in being ● Assessed and monitored ● There will be an increase in (Goal Met)
After 2 hours of effective
cystic fibrosis, I was experienced by the patient respiratory and heart rate the respiratory and heart
nursing interventions, the After 2 hours of effective
born terminal. My because cystic fibrosis is caused against normal. rate as a way of
patient will be able to: nursing interventions, the
lungs are about fifty by a mutation in the cystic compensation for early
patient was able to show
percent function right fibrosis transmembrane a. to show diminished hypoxia.
diminished nasal flaring
now,” as verbalized by conductance regulator (CFTR) nasal flaring and ● Monitored patient for signs ● Respiratory acidosis
and reduced use of
the patient. gene. The CFTR protein reduced use of of respiratory acidosis. indicates hypercapnia
accessory muscle during
produced by this gene regulates accessory muscle which can cause effects
breathing and has an
OBJECTIVE the movement of chloride and during breathing; and such as headaches,
increased oxygen
● Labored breathing sodium ions across epithelial cell b. increased oxygen shortness of breath,
saturation of 68% from
● Crackles heard on membranes.When mutations saturation from 65- dizziness, and fatigue, as
63%.
both lungs upon occur in one or both copies of 70% well as serious
auscultation the gene, ion transport is complications such as
● Nasal flaring, use defective, and results in a seizures or loss of
of accessory buildup of thick mucus consciousness.
● Monitored ABGs and
muscles, and throughout the body, leading to ● To detect for signs of
oxygen saturation as
restlessness noted respiratory insufficiency. respiratory failure.
indicated.
● Dyspnea A combination of decreased
● Assessed for changes in
● Tachypnea mucociliary clearance and an LTO: ● Patient will adapt their
respiratory status such as
● pH: 7.35 altered ion transport allow for
cyanosis, pallor, changes in breathing pattern over time LTO:
bacterial colonization of the After 72 hours of effective
● PaCO: 2-46 the level of consciousness, to facilitate gas exchange. (Goal Met)
respiratory tract. Bacterial nursing interventions, the
mmHg labored breathing and Abnormalities may indicate
patient will be able to: After 72 hours of effective
● RR: 26bpm colonization cause an respiratory compromise,
tachypnea. nursing interventions, the

28
● SpO2: 63% overwhelming inflammatory a. show no signs of hypercarbia, or hypoxia. patient was able to show
● Capilliary refill response. Ultimately, chronic respiratory acidosis; ● Stress and anxiety can no signs of respiratory
within 1-2 infection and this repetitive and ● Assessed the level of stress function as a catalyst in acidosis and reported a
seconds inflammatory response b. report diminished and anxiety. worsening breathing. diminish episodes of cold
cause severe lung damage like episodes of cold and Anxiety over the inability and clammy extremities
NURSING cysts (fluid-filled sacs) that clammy extremities to breathe makes breathing indicating an improve
DIAGNOSIS: reduce the surface area for the resulting from poor worse and therefore peripheral perfusion.
gaseous exchange, peripheral perfusion exacerbates impaired gas
Impaired Gas and fibrosis (scar tissue) in the exchange.
Exchange related to lungs, leading to impaired gas
decrease surface area exchange. Tx:
for gas exchange and ● Respiratory problems may
loss of lung function REFERENCE/S: have wheezes, crackles, or
as evidenced by Brown, S. D., White, R., & sound diminished. Changes
Tx:
crackles on both lungs Tobin, P. (2017). Keep or worsening in these lung
them breathing. Journal of ● Auscultated lung sounds
and shortness of sounds may indicate a
the American Academy of every 2 hours and listened
breath decline in ventilation.
Physician Assistants, for adventitious breath
30(5), 23–27. ● To maximize lung
sounds.
https://doi.org/10.1097/01 expansion. High Fowler’s
.jaa.0000515540.36581.9 position promotes lung
2 expansion and decreases
airway collapse, dyspnea,
‌ ● Placed patient in a high
and breath work through
Fowler’s position.
gravity.
● To help with the breathing
of the patient.
● Medications such as
mucolytics and
bronchodilators can help

29
 treat the patient’s
symptoms.
● Administered oxygen ● Ambulation helps with
therapy as indicated. lung expansion and
● Administered medications promotes deep breathing.
as ordered.

● Assisted and encouraged Edx:

the patient to ambulate as ● Early recognition and
tolerated. intervention can make a big
difference in patient’s
outcome. Taking action
may reduce the number of
hospital visits and
emergencies.
Edx:
● To help strengthen the
● Explained to the patient and
patient’s diaphragm and
significant others early signs
help improve the efficiency
of decreased oxygenation
of ventilation, decrease the
and interventions to take.
work for breathing and
improve gas exchange.
● Activity increases oxygen
needs and should be paced
● Instructed patient on how to appropriately to avoid
perform diaphragmatic fatigue.
breathing. ● Dehydration, stress, and
strenuous physical activity

30
 are some of the activities
that the patient should
monitor that could
precipitate cold clammy
● Instructed patient to space extremities. If these
activities based on activities are not well
respiratory tolerance. monitored it can lead to
shock or organ failure.
● Advised patient to monitor ● Watching out for signs and
activities that precipitate symptoms of respiratory
cold clammy extremities. acidosis will help avoid the
symptoms to get
progressively worse and
become life-threatening.
● To provide immediate
intervention to the problem
and avoid deterioration of
the condition.
● Knowledge about the use,
● Advised patient to watch out troubleshooting,
for signs and symptoms of precautions, and storage of
respiratory acidosis such as the oxygen delivery system
shortness of breath, fatigue, promotes safety.
and headache.

● Instructed patient when to

seek for prompt medical
attention.

31
 ● Explained to the patient and
family about the proper use
of oxygen therapy at home.

NCP 3: Risk for Aspiration related to the presence of secretions and bronchospasm
ASSESSMENT EXPLANATION OF THE OBJECTIVES NURSING INTERVENTIONS RATIONALE EVALUATION
PROBLEM

SUBJECTIVE: As shown In the STO: Dx: Dx: STO:

“Cystic fibrosis, as you pathophysiology because of the Within 3-4 hours of nursing ● Assessed level of ● The primary risk factor of (Goal Met)
know is a genetic airway inflammation and intervention, patient will be able consciousness. aspiration is decreased Within 3-4 hours of nursing
disease that basically production of secretions patient to verbalized on the level of consciousness. intervention, patient was
makes my body produce is breathing in a foreign object understanding on the possible ● Signs of aspiration should able to demonstrate proper
a sh** ton of mucus,” as such as foods or liquids into the causes of aspiration such as be identified as soon as tube feeding.
● Assessed respiratory rate,
stated by the patient. trachea and lungs and happens bronchial spasms and chronic possible to prevent further
depth, and effort and noted
when protective reflexes are respiratory or airway irritation. aspiration and to initiate LTO:
any signs of aspiration such
OBJECTIVE: reduced or jeopardized and can treatment that can be life- (Goal Met)
as dyspnea, cough, cyanosis.
● Coughing lead to the risk for aspiration. saving. Within 24-72 hours of
● Throat clearing LTO: ● To know if patient needs nursing intervention, the
● Assessed current level of
Within 24-72 hours of nursing more knowledge about it. patient was able to identify
understanding about the risk
NURSING REFERENCE/S: intervention, the patient will be risks of gastrointestinal
of aspiration
DIAGNOSIS: Risk for Aspiration – Nursing able to demonstrate ways of Tx: tube placement.
Diagnosis & Care Plan. managing/preventing risk for Tx: ● Ingesting food and fluids
Risk for Aspiration (2016, October 25). aspiration. ● Offered liquids after food is together decreases
Nurseslabs. swallowing difficulties.
related to the presence eaten.
https://nurseslabs.com/ris
of secretions and ● Upright positioning

32
bronchospasm k-for-aspiration/ reduces aspiration by
decreasing reflux of gastric
‌ ● Elevated the head of bed to contents.
30 to 45 degrees while
feeding the patient and for 30
to 45 minutes afterward if
feeding is intermittent. EDx:
● To allow patient to do the
EDx: exercises properly.
● Taught patient on how to
perform efficient coughing
exercises and breathing
● Upright positioning
techniques.
decreases the risk for
● Educated the patient and
aspiration.
family the need for proper
● Information helps in
positioning.
appropriate assessment of
● Instructed in signs and
high-risk situations and
symptoms of aspiration.
determination of when to
call for further evaluation.
● Clinical safety of patient
between visits is a primary
goal of home care nursing.

● Instructed patient and

significant others on ways on
how to manage episodes of
aspiration like deep ● To let the patient and
breathing, back tapping. significant others report if
there are signs and

33
● Instructed the patient and symptoms that is related to
significant others when to aspiration.
seek prompt medical ● To have plans and have
attention. interventions on it.

● Established emergency and

contingency plans for care of
patient.

34
NCP 4: Imbalanced Nutrition: Less than body requirements related to chronic malabsorption as evidenced by weight is 20% below the ideal
ASSESSMENT EXPLANATION OF THE OBJECTIVES NURSING INTERVENTIONS RATIONALE EVALUATION
PROBLEM

SUBJECTIVE: As shown in the STO: Dx: Dx: STO:

“To any of you girls out pathophysiology, in cystic ● Assessed the skin’s color, ● Malnutrition can be (Goal Met)
there wishing you could fibrosis, there are thick mucus After 2 hours of effective integrity, and turgor. associated with an
eat 5,000 calories a day generated caused by an abnormal nursing interventions, the alteration in skin integrity. After 2 hours of effective
and not gain a pound, gene (cystic fibrosis patient will be able to: ● Monitored for changes in ● Increasing trends in weight nursing interventions, the
I’ll trade you any day,” transmembrane conductance weight and appetite. and appetite accompany patient was able to
a. understand the understand the importance
as verbalized by the gene) the resolution of
importance of having of small frequent feedings
patient. pulmonary exacerbations.
that clogs the ducts (tubes) small frequent feedings for proper absorption of
● Adjustments in pancreatic
leading in and out of the to facilitate proper food as evidenced by
OBJECTIVE: enzyme supplementation
pancreas, liver, and intestines. absorption and “having small frequent
● Weight 20% below ● Assessed the abdomen for are warranted with the
These blockages mean the digestion of food; and feedings every day can help
the ideal bloating, fullness, bowel signs or symptoms of
pancreas cannot push enough b. verbalize proper me have proper absorption
● Excessive weight sounds, or palpable stool malabsorption.
enzymes into the intestines for selection of foods or of the foods that I eat”, as
loss and loss of mass. ● Foul-smelling, large and
food to be digested properly.This meals that will enhance verbalized by the patient.
subcutaneous tissue greasy stools suggests that
may cause a decrease in the weight gain such as The patient was also able to
noted the digestive system is not
secretion of enzymes from the high caloric high fat verbalized proper selection
● Dry hair and skin ● Monitored stool patterns for breaking down food
pancreas that normally help diet. of foods or meals that will
● Pale oral mucusa frequency, odor, adequately.
● Large and greasy digest food. A person with cystic ● Correct placement of the enhance weight gain as
consistency, and the
stool fibrosis has trouble absorbing tube should be confirmed evidenced by “I should take
LTO: presence of oil or grease.
proteins, fats, and vitamins A, D, to have a successful these
NURSING E, and K. After 1 week of effective administration of enteral vitamins with pancreatic
● Checked insertion site of G-
DIAGNOSIS: nursing interventions, the feed. enzymes to be absorbed
Without enzymes, maldigestion tube at the abdominal wall
patient will be able to: properly and I should eat
and malabsorption of food can and observed for abdominal
high fat, high calorie, high

35
Imbalanced Nutrition: occur causing imbalanced a. report weight gain of at pain or discomfort. Tx: fiber, have sufficient intake
Less than body nutrition that is less than the least 2.2 pounds; and ● Client with cystic fibrosis of salty foods and have a lot
requirements related to body requirements. b. maintain adequate Tx: requires pancreatic enzyme of water to help improve
chronic malabsorption nutritional status by ● Administered pancreatic supplementation to my nutritional status,” as
as evidenced by weight following enzymes before all meals adequately digest food verbalized by the patient.
is 20% below the ideal recommended diet and snack containing fat or containing fat or protein.
REFERENCE/S: plans. protein as ordered. ● Fat-soluble vitamin deficits LTO:
Cystic Fibrosis. (2021). are common in cystic (Goal Partially Met)
Hopkinsmedicine.org. fibrosis because of fat
https://www.hopkinsmedi After 1 week of effective
● Administered all fat-soluble malabsorption. Vitamin
cine.org/health/conditions nursing interventions, the
-and-diseases/cystic- vitamins with meals and supplements must be taken
enzymes. with pancreatic enzymes to patient was not able to
fibrosis
be absorbed. achieve 2.2 pounds but had
‌ ● For better use of G-tubes weight gain of 1.6 pounds.
and administrations of She also maintained
medications and for safe adequate nutritional status
‌ food delivery. by following her
● Multidisciplinary input and recommended diet.
collaboration will optimize
● Supervised feedings and
therapeutic interventions.
used the "teach-back"
method on how to use the G-
tube. Edx:
● Small frequent meals help
in proper food absorption.
● Collaborated with a
registered dietician in
obtaining a full nutritional
evaluation. ● Client with cystic fibrosis
has increased caloric needs
as much as 1.2 to 1.5 of the

36
Edx: daily recommended
● Encouraged small frequent amounts.
meals and taught its ● Meal planning on the daily
importance for proper food food intake help the patient
absorption. toward achieving optimum
● Advised patient on high body weight gain and
caloric high fat meal nutritional status.
options.
● Chronic constipation can
result from mucus
secretion and dehydration
in the intestinal lumen.
● Instructed on proper diet and
● Hyponatremic dehydration
meal planning strategies to
and salt loss occur easily in
help maintain optimum body
the client with cystic
weight.
fibrosis as sequelae to
● Encouraged liberal hydration
cystic fibrosis defect in the
and high fiber intake.
sweat glands.
● To avoid possible mistakes
in delivering medication
● Encouraged ample use of
and food in the G-tube.
salt or salty food intake.
● To be able to have a
successful pharmacologic
treatment.

● Taught patient and

significant others how to
prepare and administer

37
 medication and formula in
G-tube.
● Taught patient about the ● To provide proper
importance of taking her intervention and prevent
medications and vitamins deterioration of the
and emphasized the condition.
importance of vitamin
supplements that must be
taken with pancreatic
enzymes to be absorbed.
● Instructed patient and
significant others the things
that needs to be reported to
the nurse and physician
concerning patient’s
nutritional status like
vomiting, diarrhea that lasts
more than 2 days and
drainage from the tube site.

38
NCP 5: Risk for Infection related to presence of G-tube on the left lower abdomen
ASSESSMENT EXPLANATION OF THE OBJECTIVES NURSING INTERVENTIONS RATIONALE EVALUATION
PROBLEM

SUBJECTIVE: As shown in the STO: Dx: Dx: STO:

“She left me a bunch of pathophysiology, the patient After 2 hours of effective ● Assessed for the general ● Fever, cough and malaise (Goal Met)
pills, some G-Juice. developed maldigestion and nursing interventions, the signs and symptoms of are one of the general (Goal Met)
Yum! that’s a liquid malabsorption of food because patient will be able to: infection such as fever, symptoms of infection.
nutrition that goes of the decrease secretion of a. understand the cough, malaise. After 2 hours of effective
straight to my stomach enzymes from the pancreas that importance of having 6 ● Monitored vital signs nursing interventions, the
through my G-tube,” as help for food digestion. Because feet apart from other especially temperature. ● High temperature can patient was able to
verbalized by the of this, the patient was inserted patients experiencing indicate infection. understand the importance
patient. with G-tube on her left lower the same condition and ● Assessed ostomy site for any ● Redness, swelling, unusual of having 6 feet apart from
abdomen for additional delivery the importance of signs of infection like smell and pain in the other patients experiencing
of nutrients in her body but this using personal redness, swelling, unusual ostomy site indicate the same condition and the
OBJECTIVE: g-tube can introduce bacteria or protective equipment smell and pain. infection. importance of using
● Presence g-tube viruses into the body that can when going outside the personal protective
attached to lower Tx: Tx: equipment when going
make her to be at risk for room;
left abdomen, open infection. b. demonstrate a outside the room as
wound and exposed ● Washed the skin around the  To avoid infection, skin evidenced by “I should
meticulous hand
when feeding ostomy site with mild soap irritation and breakdown always have a 6 feet
washing technique;
● Decreased skin REFERENCE/S: and warm water twice a day around the G-tube. distance from other patients
and
integrity around the Stanford Health Care. (2017, and kept skin clean and dry. with the same condition and
c. demonstrate on how to
stoma September). ● Changed the dressing once a  Changing the dressing wearing a mask and gloves
properly perform
Stanfordhealthcare.org. day and when it gets dirty to every day prevent the is important when I want or
https://stanfordhealthcare. ostomy care.
NURSING protect the ostomy site from build-up of microorganism need to go outside my
DIAGNOSIS: org/medical-treatments/f/f infection or microorganism. and lessen the risk for room,” as verbalized by the
eeding-tube/complications ● Washed hands properly
.html infection. patient. The patient was
Risk for Infection before and after cleaning the also able to demonstrate a

39
 ostomy site and handling the  To avoid transmission of meticulous hand washing
related to presence of G- ‌ LTO: patient. technique and how to
microorganisms.
tube on the left lower After 24 hours of effective properly perform ostomy
abdomen ‌ nursing interventions, the EDx: care.
patient will be able to:
‌  Instructed to always have 6
will be able to:
‌ feet apart from other LTO:
a. maintain no signs of EDx:
patients with cystic fibrosis. (Goal Not Met)
infections such as  People with cystic fibrosis
redness, swelling, and After 24 hours of effective
should never meet each other,
pain in the ostomy site; nursing interventions, the
as they carry bacteria within
and  Emphasized the importance patient developed infection
their lungs that could be
b. keep the ostomy site of using personal protective in her G-tube resulting in
harmful to each other.
clean and dry to help equipment when going surgery to avoid further
 To prevent or lessen the risk
guard against skin outside the room. complications.
of having different bacteria
irritation and  Emphasized the importance
and viruses.
infections. of proper handwashing and
use of gloves when handling
or disposing of tissues,
 Prevents spread of fluid-borne
sputum containers, and
pathogens.
when going outside.
 Taught on how to properly
perform ostomy care.
 Emphasized the importance
of maintaining the ostomy
site clean and dry to prevent
skin irritation and infection.
 Instructed on which
symptoms of infection to  To prevent skin irritation and
watch out and report like infection around the ostomy

40
redness and swelling in the site.
ostomy site.  To lessen the risk of
developing infection with the
G-tube.

 To immediately provide
intervention.

41
C. Discharge Plan
Health Teaching
Diet/Nutrition 1. Instructed patient and significant others to consume or
prepare a balanced diet with plenty of fruits and vegetables,
whole grains, dairy products, and protein.
2. Encourage patient to consume foods with nutrients such as
protein, iron, salt, zinc, fiber, antioxidants and calcium.
3. Encouraged patient to maintain a good nutritional status
which can help in improving her well-being, keep symptoms
under control, and fight infections.
4. Instructed to take vitamin supplements and enzyme
supplements as prescribed.
Activity 1. Encouraged patient to take adequate rest.
2. Instructed patient to follow the exercise plan as doing
physical activities can help loosen secretions in the airways
and lungs, and help her breathe more easily.
Medication 1. Instructed patient to continue taking medications as directed.
2. Orient the patient about the drug’s names, effects, dosage,
frequency, route, and adverse reactions.
3. Instructed to carry a medicine list in cases of an emergency.
4. Instructed patient and significant others to report immediately
if unusual reactions take place.
Treatment 1. Emphasized on the importance of follow-up checkups.

Other 1. Provide teaching and instruction manual about equipment.

2. Instructed to be careful around other people with cystic
fibrosis since the patient could end up catching other’s
bacteria.
3. Instructed to observe infection prevention by wearing mask,
washing hands, and wearing gloves.

42
XVI. Learning Insights
A. ALUAD, Rosella G.
As I read about cystic fibrosis, I learned what Cystic Fibrosis means is and what are the
causes. In my own understanding this disease is a genetic condition that causes long-term lung
infections and gradually reduces one's ability to breathe. Furthermore, I was able to know that
mucus buildup hinders the release of digestive enzymes that aid in the body's absorption of food
and vital nutrients, leading in malnutrition and poor growth. The thick mucus in the liver might
clog the bile duct, resulting in liver disease. Thus, the types of symptoms and their severity might
vary greatly from one person to the next. Many factors, including your age when you are
diagnosed, might influence a person's health and the course of the condition. I also became aware
that diabetes, thin, weakening bones (osteoporosis), infertility in males, and liver problems are all
possible complications of the disease as I do my readings. I learned the specific signs and
symptoms and how to address them, might as well the diagnostic procedures, treatment, surgical
managements, and most especially precautionary measures. Nurses’ roles in caring for a patient
with cystic fibrosis includes relieving immediate respiratory distress, maintaining adequate
oxygenation, remaining free from infection, improving nutritional status, relieving anxiety.
Nurses take a vital role as well in assessing and giving adequate health teaching and emotional
support to patients with Cystic Fibrosis.

B. CALIXTO, Loraine Ruth P.

As I watch the movie I felt sad because at a young age she already had a difficult disease and
it melt my heart to see that even though she suffers from this disease she still laughs, smile and play. I
then saw how the patient was struggling. With a diagnosis of cystic fibrosis, it is really heartbreaking
since the only cure is to get a lung transplant. With these, it helped me grow as a student because I was
able to learn about new things and how it gets worse or complicated. Establishing rapport to one's own
patient and to his/her significant other/s is really important because it helps you have an insight on
what is going on and how you will approach, plan, and give care and treatment.

C. DULAWAN, Juliene Grace Talacca G.

As we all know, cystic fibrosis is an inherited disorder that causes severe damage to the lungs,
digestive tract and other organs that require several surgeries. I have learned that there is much to
know about this disease and we’re lucky that were able to breathe fresh air and not from a borrowed
one. People with CF tends to break down but still needs emotional support from their loved ones even
though interaction is limited to make the condition easier to live with.

D. GAMONNAC, Maria Crystal Quinn F.

It has always been emphasized and reiterated that the care a nurse provides is specifically
unique to each patient. Although having case presentations is primarily aimed at helping us better
ourselves in understanding certain conditions and honing our skills in analysing and understanding
certain situation, this as well remind me that nursing is the profession that treats patients as people and
not just a collection of body systems, with some malfunction and disease.

Every time we do a case analysis, I try to imagine and play out scenarios in my head how we
would have approached the actual patient in an actual setting. This particular case analysis somewhat
scared me into wanting to read a lot and exert more effort in understanding concepts and familiarizing
myself with certain medical terms and such, in hopes of being actually able to help the best way I can,
when soon faced with actual patients with such conditions.

E. LANGBAYAN, Zaskhia C.
Watching the movie, the second time made me understand more what the movie is all about
even though this time is only for educational purpose. As I have watched and read about Cystic
Fibrosis for me it is one of the complicated illness. Every move and action being done must be taken
in caution where on this part the nurses role is very much in need to always remind the patient on what
to do and by making sure the everyday management is met.
On my part on the case presentation, I did the drug study. There are a lot of drugs that were
mentioned but I only chose the drugs I think is common and is related on the case of the patient. It is a
great learning experience since I was able to gain a little set of knowledge.

43
F. LAO-ING, Jaimelyn S.
On this case study I was able to work on the 13 areas of assessment. I have learned many
things while doing research regarding this case study like the abnormal symptoms of cystic fibrosis.
while doing my part I gained a lot of knowledge about this disease on how cystic fibrosis patients
should not be in the same space as each other. People with CF get infections that people without CF
do not catch, and they’re especially likely to transmit those germs to others with the disease. This film
about CF will help to raise awareness of the real impact of cystic fibrosis. Lastly as a student nurse we
should have appropriate knowledge regarding this disease in order for us to be aware and to prevent it
from reoccurring.

G. LUPAE, Frannie D.
The case was given to us via movie. It was a new method for my group so it was quite a
challenge but I think that this method is more interesting and a lot easier as compared to written cases.
My part was on the health assessment so I had to observe the details and figure out the reasons behind
it. Through this case, I learned the basic concepts of Cystic Fibrosis. I learned about the common
assessments of a CF patient including the signs and symptoms and the common problems that they
encounter. The movie helped us come up with our group output but I feel that there are still concepts
lacking on our output especially on the health assessment part. I hope that our knowledge will be
supplemented by our CI during our presentation.

H. MAGAT, Camil Joy R.

With this case presentation, I was able to enhance my skills as an individual and work with
others. The case was given through a movie and understanding and making a case report for a patient
we have not handled is really a challenge, but through this, I learned the importance of teamwork
where we were able to support and help each other out. Also, I gained knowledge and further
understanding about the condition of the patient which is Cystic Fibrosis like its pathophysiology,
diagnostic and nursing care management or treatment. In addition, our patience and communication as
a group were also enhanced throughout the making of this case study. I believe that these experiences
of us and knowledge that we gained will be a great help to our future profession.

I. MATEO, Maria Diana A.

With this case study, I got an opportunity to gain new insights and a better understanding of
cystic fibrosis through a movie. In doing our case study, we were given the freedom to choose what
part of we wanted to do. I chose to do our nursing care plan and prioritization together with my other
groupmate. While doing these parts, I am gaining knowledge about the different nursing interventions
to some of the nursing diagnosis associated with cystic fibrosis. I also learned that as a student nurse, I
should always be prepared in rendering or providing care for the patients with cystic fibrosis to help
them ease the symptoms that make them uncomfortable or suffer. It is also important for us student
nurses to have appropriate knowledge regarding this condition in order for us to be aware, as well as
raise awareness, to the public to help avoid or manage such condition.

J. PIT-OG, Jhayrelle Annesy I.

This case study was as refreshing as it was frustrating. Refreshing in a way that
we get to watch our ‘patient’ live out her life with cystic fibrosis and it showed me that for people
with this disease, every breath is a struggle, a triumph, and a painful reminder that it may be the last,
that is the frustrating bit about this case study and through the articles and researches I have read to
plot out a comprehensive pathophysiology, a single mutation in the gene has cascading effects on the
body.

K. REIJNDERS, Hazel Joy B.

Through this case study, I was able to learn more about Cystic Fibrosis. Though it is not the
usual way on how we go about making the case presentation, I do appreciate it because I was able to
grasp on how patients with CF live their lives and how nurses care for them even though there was not
much exposure on the movie. Although we had a visual representation of the patient it was still a
challenge since we were not able to assess the patient physically, so I had to widen my imagination
and read about the disease to help me in accomplishing my part.

44
45
XVII. List of References

5 Cystic Fibrosis Nursing Care Plans. (2017, April 19). Nurseslabs. https://nurseslabs.com/cystic-fibrosis-
nursing-care-plans/

BNS Staff. (2020, October 2). Trikafta. Cystic Fibrosis News Today.
https://cysticfibrosisnewstoday.com/trikafta/

Birket, S. E., & Rowe, S. M. (2019). Revealing the molecular signaling pathways of mucus stasis in cystic
fibrosis. Journal of Clinical Investigation, 129(10), 4089–4090. https://doi.org/10.1172/jci131652

Brown, S. D., White, R., & Tobin, P. (2017). Keep them breathing. Journal of the American Academy of
Physician Assistants, 30(5), 23–27. https://doi.org/10.1097/01.jaa.0000515540.36581.92

Burns, J. L., & Rolain, J.-M. (2014). Culture-based diagnostic microbiology in cystic fibrosis: Can we simplify
the complexity? Journal of Cystic Fibrosis, 13(1), 1–9. https://doi.org/10.1016/j.jcf.2013.09.004

Calgary Guide. (2014). The Calgary Guide to Understanding Disease. https://calgaryguide.ucalgary.ca/?

fbclid=IwAR2BsWcT3mX9W9dds6qC4cRTCPLKPri3zByjAVK5sXrPzmlR6Q23qpJ0q-Q

Callaghan, B. D., Hoo, A. F., Dinwiddie, R., Balfour-Lynn, I. M., & Carr, S. B. (2005). Growth and lung
function in Asian patients with cystic fibrosis. Archives of Disease in Childhood, 90(10), 1029–1032.
https://doi.org/10.1136/adc.2004.067264

Cystic Fibrosis (Adults) | Michigan Medicine. (2021). Uofmhealth.org.

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XVIII. Appendices

48
 Appendix A
Approval/Request Letter

Judith A. Layao
Area Head

Thru: Juliet V. Avena

Clinical Coordinator

Dear Ma’am,
Greetings!
We, the Level IV Section C Group 2E, would like to reserve the case with a diagnosis of Cystic
Fibrosis for our case presentation this first semester of school year 2021-2022. This case was
obtained from a movie entitled “Five Feet Apart.”

We have selected this case to further enhance our knowledge and management regarding this
problem that we have chosen.

Thank you very much for your kind consideration and God bless!

Respectfully yours,

_______________________
Rosella G. Aluad _______________________
Maria Diana A. Mateo
_______________________
Loraine Ruth P. Calixto _______________________
Jhayrelle Annesy I. Pit-og
_______________________
Juliene Grace Talacca G. Dulawan _______________________
Hazel Joy B. Reijnders
_______________________
Maria Crystal Quinn F. Gamonnac

_______________________
Zaskhia C. Langbayan

_______________________
Jaimelyn S. Lao-ing

Noted by:

_______________________
Clinical Instructor

_______________________
Frannie D. Lupae

_______________________
Camil Joy R. Magat

49