1st Rot Final CP

A Case Study on
Cystic Fibrosis
In Partial Fulfillment of the Requirements in
RT-124 Clinical Education 1
PEDIATRIC INTENSIVE RESPIRATORY CARE
ROTATION
Submitted to:
GUIA LAUREN L. Tarranza, RTRP
Clinical Instructor
Submitted by:
Agustin, Joannes Lorenz
Azarcon, Ryan
Boca, Princess Yliezah
Lee, Russel
Pumares, Denise Louise
Refuerzo, Audrey Lou
Sabatin, Christian Miguel
Servinio, Ionna Gabrielle
Testa, Tresha
BSRT 4C - Group 9
August 9, 2022
TABLE OF CONTENTS
I. Cover Page ..................................................................................................1

II. Table of Contents .......................................................................................2
III. Introduction ...............................................................................................4
IV. Objectives .............................................................................................….6
a. General Objectives............................................................................…....6
b.Specific Objectives ...................................................................................6
V. Definition of Diagnosis .............................................................................7
VI. Personal Data ......................................................................................…..9
a. Biographical Data............................................................................….......9
b. Medical Data ......................................................................…....................9
VII. Patient History ..........................................................…..........................10
a. Past Health History....................................................…...........................10
b. Maternal Health History.............................................…...........................10
c. Present Health History..............................................…............................10
d. Genogram ....................................................................................….........11
VIII.Assessment ...........................................................................................13
a. Physical.......................................................................…......................... 13
i. General Survey..................................................…………….......................13
ii. Vital Signs..............................................…………...................…...............13
iii. Quiet examination ....................................…………................................13
iv. Hands-on examination .......................................…………......................15
b. Developmental Task...................................................…..........................18
IX. Anatomy and Physiology .......................................................................23
X. Pathophysiology .........................................................................…….....27
a. Factors ........................................................…......................…................27
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b. Diagram................................…................................................................31
c. Narrative...................................................…............................................33
XI. Diagnostics .....................................................…....................................36
XII.Drug Study ..............................................................................................45
XIII. Respiratory Therapy Care Plan ........…...............................................57
XIV. Pulmonary Rehabilitation ...............................................................….90
XV. Prognosis .....................................................................................…......93
XVI. References ............................................................................................94
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INTRODUCTION
Pediatric intensive care is recommended whenever pediatric patients

experience hemodynamic instability that require attentive monitoring of vital
signs, invasive hemodynamic monitoring, quick administration of medication,
and respiratory support. This type of care allows pediatric patients to have an
improved quality of care and better prognosis (Seifu, et al., 2022). A pediatric
intensive care unit (PICU) is a hospital unit wherein children with life-
threatening status requiring pediatric care are tended to (Tazebew, et al.,
2019)
The major essence of pediatric care is to decrease mortality by closely
monitoring and developing treatment options for severely ill pediatric patients
who are regarded as a high risk for death. Analyzing the results of medical
treatments will help determine the therapeutic intervention's success (Seifu, et
al., 2022). In addition, the performance of medical practitioners stationed in
PICUs, appropriate medical equipment, a clean environment to treat patients,
and a systemic organization in allocating resources are necessary factors in
providing optimal pediatric intensive care (Bjorklund, 2022).
In ancient Europe, children affected with cystic fibrosis were thought to
be cursed by witches to die with a short lifespan. The folklore was, "Woe to
the child who tastes salty from a kiss on the brow, for he is cursed and soon
will die." This folklore was mostly referring to the salty skin that is an evident
sign of cystic fibrosis, an illness without known cause or cure (Yu & Sharma,
2021). Autosomal recessive mutations of the CF transmembrane conductance
regulator (CFTR) gene, which codes for the CFTR protein, cause the fatal
monogenic condition called Cystic Fibrosis (CF) (Bergeron & Cantin, 2019). It
is a fatal disorder that most often leads to chronic sinopulmonary infections
and pancreatic insufficiency, with end-stage lung disease as its most common
cause of mortality.
Meconium ileus, persistent neonatal jaundice, or an early lung infection
can all occur in newborns with CF. Failure to thrive and inadequate weight
gain, anemia, boys' undescended testicles, recurrent sinopulmonary
infections, and distal intestinal obstructive syndrome with or without
pancreatic insufficiency are all possible symptoms in infants and children with
cystic fibrosis (CF). Despite the fact that individuals may not show clinical
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signs and symptoms until later, the median age of diagnosis is 6 to 8 months
(Yu & Sharma, 2021).
In the Philippines, the Department of Health declares a disease to be
rare when it afflicts one patient in every 20,000 people in the country. Since
CF is a rare disorder and often affects the population of Caucasian heritage,
there is little to no statistics present within the Philippines. However, in an
Enhanced Newborn Screening Program published for the Philippines, the
estimated annual cases of Filipino newborns with CF born in the Philippines
rounds up to about 100 (Padilla, et al., 2022). Currently, there is no available
recorded literature for patients with cystic fibrosis in Davao City.
About 90,000 people worldwide suffer from Cystic Fibrosis (CF), which
is the most prevalent fatal genetic condition with severe life-limiting symptoms
(Lee JA, et al., 2021). Previously, CF has been accepted to affect 1/2500 live
births for people with European descent. However, this data from the past
seemed to be lower. In today's scenario, the epidemiology of CY rounds up to
about 1/3000 to 1/6000 (L'Hostis, et al., 2020)The fluctuating estimation for
CF study can be attributed to lacking or just recently developed Newborn
Bloodspot Screening (NBS) which was only implemented and continuously
developed over the last decade (Scotet, et al., 2020).
With the aforementioned information, the findings of this study will be
significant in contributing to the field of Respiratory Therapy. People in the
academe, both students and lecturers alike, will benefit in using the
accumulated information in developing care strategies for pediatric patients,
especially for those diagnosed with rare disorders like cystic fibrosis.
Moreover, practitioners in the field will be able to benefit with this study in the
sense that they will be able to start considering treatment options and therapy
care plans for patients affected with cystic fibrosis. Lastly, since cystic fibrosis
is known for its rarity, researchers can gather more information and use the
results of this study as a tool for reference in developing their studies. They
will be able to utilize the inputs in this study as a contribution for the
foundation in understanding more about the context of cystic fibrosis.
GENERAL OBJECTIVES
Following our first rotation for Pediatric Intensive Respiratory Care
(PIRC) for the first semester, the student interns of Group 9 of Bachelor of
Science in Respiratory Therapy will be able to create a thorough case
analysis about Cystic Fibrosis. This will enable them to create a more deeper
understanding about the said disorder and fortify their knowledge about the
background, causes, factors of this condition and establish their critical
thinking skills in developing respiratory care and management necessary that
can be applied in their actual practice.
SPECIFIC OBJECTIVES
In order to achieve the general objectives aforementioned, the student
interns specifically aim to:
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a. provide an introduction that presents the relevance of the Pediatric
Intensive Care rotation, which also includes a general summarization of
the disease featured, recent available date for cystic fibrosis, and the
significance of this case study;
b. define and present detailed explanation about CF from at least three
credible sources;
c. state the patient's biological and medical information by presenting
the past, maternal and present health histories, including a family
history up to three generations;
d. discuss the patient's medical history through his past and present
health histories including a family history stretched up to three
generations;
e. execute a detailed patient examination from head to toe, recording
any abnormalities noted through the manner of inspection, palpation,
percussion, and auscultation;
f. distinguish the anatomy and physiology of the organ system affected
by the disease;
g. examine the pathophysiology of CF including its etiology,
symptomatology, and risk factors;
h. list all the medications used with its brand and generic name,
classification, mode of action, dosages, indications, side and adverse
effects, drug interaction, and relevant respiratory therapy management;
i. construct three respiratory care plans; and
j. formulate the prognosis of the condition of the patient by examining
the response to the therapy and medication.
DEFINITION OF DIAGNOSIS
Cystic Fibrosis is one of the most common genetic disorders. It is an

inherited condition found in the children that affects the way salt and water
move in and out of the cells. This type of genetic condition causes the patients
respiratory, digestive, and reproductive systems to become clogged with thick,
sticky mucus. About 40,000 children and adults are affected with cystic
fibrosis in the United States. Moreover, it is an autosomal recessive caused
by mutations in the cystic fibrosis transmembrane conductance regulator
(CFTR) gene (Cystic Fibrosis Foundation, 2019). The defective gene causes
the CFTR protein to not form or work properly, resulting in abnormal flow of
salt and water across the cell, leading to dehydration of the airway surface
and thick mucus that blocks the ducts throughout the body (American
Thoracic Society, 2017).
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The primary function of CFTR is it acts as a chloride pathway,
transporting ions across the apical membranes throughout the body. It also
serves as sodium transport inhibition and bicarbonate secretion that are
essential for the pathophysiology of CFTR deficiency. Progressive lung
disease, exocrine pancreatic insufficiency leading in gastrointestinal
malabsorption, intestinal abnormalities resulting in malnutrition, decreased
development, and a number of additional signs, including sinusitis and
diabetes, characterize the cystic fibrosis phenotype (Ratjen, et al., 2020). The
severity of the illness in each individual depends on the varying organ
sensitivity and on the genetically determined residual function of the CFTR
protein. Apart from that, as a result of obstructive azoospermia, 99% of the
male patients are impotent, and 87% of patients have exocrine pancreatic
insufficiency. Moreover, the degree of severity in patients that have pulmonary
involvement also depends on other disease-modifying genes and on the
patient’s socioeconomic setting (Naehrig et al., 2017).
The progression of chronic lung and paranasal sinus illness varies
across cystic fibrosis patients and can be difficult to identify from recurring
attacks of bronchitis and/or pneumonia, particularly in preschool children.
Children who have a cough, sputum production, or wheezing for more than
three months, constant abnormal radiological findings, clubbing of fingers,
positive bacterial cultures of respiratory secretions should be tested for cystic
fibrosis even if their neonatal screening test was negative. Moreover, the
lungs and pancreas are the organs most commonly damaged by cystic
fibrosis, which can cause respiratory and digestive problems. Mucus can still
hold bacteria in a person with cystic fibrosis, but it has problems moving out of
the lungs. As a result, bacteria remain in the lungs, and can cause serious
infections (Naehrig et al., 2017).
When a baby is diagnosed with cystic fibrosis, he or she inherits two
cystic fibrosis genes, one from each parent. A person who carries only one
cystic fibrosis gene is considered healthy and a “carrier” of the disease. If both
parents have the cystic fibrosis gene, they each have one copy of the
defective gene, but are not affected by the disease. Their children have a 25
percent chance of inheriting both defective copies and having cystic fibrosis,
50 percent chance of inheriting one defective copy and being a carrier or a 25
percent of not having cystic fibrosis and not carrying the gene (Boston
Children's Hospital, 2022).
Symptoms of cystic fibrosis differ from person to person, depending on
the severity of the condition. For example, one child with cystic fibrosis may
have respiratory but no intestinal issues, whereas another child may have
both. Furthermore, the signs and symptoms of cystic fibrosis may change with
age. People with cystic fibrosis sweat two to five times the typical amount of
salt (sodium chloride). Growth delays are common, and children with cystic
fibrosis are often smaller than their peers their age. They may also experience
dyspnea and have difficulty in exercising. Rectal prolapse can occur
infrequently as a result of frequent coughing or difficult-to-pass feces. This
signifies that a portion of the rectum protrudes or sticks out through the anus.
This affects around 20% of children with cystic fibrosis. Rectal prolapse is
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sometimes the first indication of cystic fibrosis. It is rare in children without
cystic fibrosis, although it does occur (Children’s Hospital of Pittsburgh, 2018).
According to the American Thoracic Society (2017), there is no cure for
cystic fibrosis, life expectancy has steadily improved with the median survival
exceeding 45 years in the United States, and there are currently more adults
with cystic fibrosis than children. Symptoms such as repeated respiratory
infections, malnutrition, and/or male infertility are commonly used to identify
older children and adults. Even though cystic fibrosis is more common in
Caucasians, it is also present in all races and ethnicities.
PERSONAL DATA
A. Biographical Data
Name: T.L.C.
Gender: Male
Age: 13 years old
Height: 59 inches
Weight: 70.54 lbs (32 kg) upon admission
BMI: Underweight
Birth date: March 22, 2009
Birth Delivery: Vaginal Delivery
Place of Birth: Davao City
Nationality: Filipino-German
Religion: Roman Catholic
Mother’s Occupation: Housewife
B. Clinical Data
Date of Admission: July 29, 2022 (Due to pulmonary
exacerbation)
Time of Admission: 11:24 AM
Manner of Admission: Via wheelchair
Hospital: San Pedro Hospital, Davao City
Ward: Pediatric Intensive Care Unit, Room 2
Admitting Physician: Dr. Guia Lauren Tarranza, RTRP, MD
Chief Complaint: According to the mother: Worsening
dyspnea, chronic
cough with productive occasional blood-
tinged and
green sputum, fevers, anorexia
Admitting Diagnosis: Exacerbation related to recurrent pulmonary
infection
secondary to cystic fibrosis.
PATIENT HISTORY
This part will thoroughly cover the elements of history taking which
includes the past health history, maternal health history, and present health
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history, and a genogram exhibiting the patient's family relationships and
medical history. A narrative describing the genogram will follow.
A. Past Health History
Patient TLC is a thirteen-year-old male of Filipino descent. He was

born on March 22, 2009 at full-term within 38 weeks gestational age. He was
delivered vaginally to a 26-year-old mother with a normal birth weight of 2835
g (2.84 kg) and 53 cm (20.87 in) of height. At 13 days of life, he exhibited his
first lung complication with pleural effusion. At 1 month of age, he was
diagnosed with Cystic Fibrosis after a positive screen result from newborn
screening and a confirmatory CF gene mutation testing that revealed a
homozygous F508 del mutation. He was admitted two times in the last 13
years for recurrent pulmonary infections of Pseudonomas aeruginosa. As for
the patient’s dietary needs, he is not consistent with the diet required for his
condition as he only consumes the same food as his family. Patient’s bedtime
is 9:00 PM, but wakes up regularly in the middle of the night due to coughing
and medication which takes up to an hour before sleeping again. He wakes
up at 6:30 AM for school on week days and 8:00 AM on weekends. He used
to sustain his daily activities with his medical regimen that includes
prophylactic antibiotic, NSAIDs, anti-ulcer and antacid, antihistamine drugs,
pancreatic enzymes, and vitamins. However, due to financial constrictions,
the family was not able to buy the medications needed for the past two
months for patient TLC and was only able to provide the patient's usual
enzymes.
B. Maternal Health History
Patient TLC's mother was a 26-year-old primigravida. The mother's father was
diagnosed with Cystic Fibrosis. Although the patient's mother does not have
CF, she is aware that she is a carrier of the mutated gene. Her husband also
does not present any manifestations of CF, but comes from a generation with
a history of CF; genetic testing confirmed that he [husband] was also a CF-
carrier as well. Prior to her delivery, she was able to have a complete
antenatal care as planned by her obstetrician. She was also able to maintain
a healthy and balanced diet throughout her pregnancy coupled with the
necessary prenatal vitamins. At 38 weeks of gestation, the mother
encountered premature contractions and rupture of membrane. She was then
rushed immediately to San Pedro Hospital where she delivered patient TLC.
C. Present Health History
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The thirteen-year-old male was rushed to San Pedro Hospital via wheelchair
after complaints of 109, fatigue, low-grade fevers, and weight loss
accompanied with dyspnea. There was absence of other necessary
complaints in the other organ systems.
Upon admission, the patient exhibited dyspnea with chronic cough
productive of green sputum. Vital signs taken include a blood pressure of
111/63 mmHg, heart rate of 111 bpm, respiratory rate of 34, oxygen
saturation (SpO2) 92% on room air, temperature of 39°C taken orally.
Auscultation revealed remarkable coarse breath sounds as well as scattered
expiratory wheezes. Patient also has a slightly increased work of breathing
and clubbing of his fingernails. Patient has a GCS score of 12 which means
moderate level and a lethargic level of consciousness. Patient TLC was then
put on oxygen by nasal cannula initially instituted at 2 L/min and was given a
small intravenous (IV) fluid bolus.
The physician requested for Chest X-ray and results displayed mucous
plugging, peribronchial thickening, and ground-glass opacities consistent with
consolidation in the alveoli of the lungs. Because the patient has a known
diagnosis of cystic fibrosis and a previous Pseudonomas infection, an oral
antipseudomonal fluoroquinolone (Ciprofloxacin) was administered with a
dosage of 20 ml/kg in a 2-repeat dose taken every 12 hours.
Patient had clubbing on his fingers and was submitted for ABG. His
arterial blood gas test results show partially compensated metabolic alkalosis
with uncorrected hypoxemia. To address this, patient was given 4ml of 7%
inhaled hypertonic saline, administered twice daily and 12 hours apart, in
order to improve sputum properties and facilitate mucociliary clearance.
Bronchodilator (Salbutamol) dosing 2.5 mg was given prior to nebulization to
facilitate airway dilation.
Sputum obtained from the patient was sent to a culture test which
confirmed the presence of Pseudomonas aeruginosa. Following the
aforementioned results and after confirmation from further examinations, Dr.
Tarranza diagnosed Patient TLC with exacerbation related to recurrent
pulmonary infection secondary to cystic fibrosis.
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D. Genogram
Genogram Narrative
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Patient TLC is a 13 years old who has been diagnosed with Cystic
Fibrosis since his first month of age. Specifically, gene testing revealed a
homozygous F508 del mutation. Last July 19, 2022, the patient was admitted
to the hospital for an acute pulmonary exacerbation due to recurrent infection
caused by Cystic Fibrosis.
Tracking the patient's genogram, the paternal side revealed a history of
cystic fibrosis and diabetes. The grandfather was of German heritage, and
was diagnosed with cystic fibrosis all throughout his life. Although the disease
was managed, he died from complications due to the diagnosis at 61 years
old. While the grandmother is also of German heritage, she was diagnosed
with diabetes and is currently on insulin maintenance. The father of the patient
is a known smoker and is a known carrier of the gene cystic fibrosis confirmed
through genetic testing.
For the maternal side, it shows a history of hypertension and cystic
fibrosis. The grandfather is a smoker with a record of 2 packs a day.
Moreover, the grandmother is diagnosed with hypertension and is given anti-
hypertensive drugs for maintenance. Additionally, she is also diagnosed with
cystic fibrosis and is maintaining a lifestyle with the recommended diet and
medications for her diagnosis. The mother of the patient is not positive of
cystic fibrosis, however genetic testing revealed her to carry the defective
CFTR gene.
As per this family history, it should be noted that the parental German heritage
(Caucasian) is a predisposing factor for the diagnosis of the patient.
Moreover, both the parental and maternal side carry the defective CFTR gene
which serves as strong evidence for the diagnosis of the patient. It should also
be taken into consideration that despite the prevalence of CF cases of the
family, the patient was unable to maintain the daily regimen suggested to him
due to financial constrictions. Despite being familiar with how the disease
works and progresses, certain limitations are imposed because of the inability
to maintain the medications, treatment, enzymes, and overall lifestyle needed
for the prevention of exacerbations of the said disease.
PATIENT ASSESSMENT
A. PHYSICAL ASSESSMENT
Date: August 6, 2022, 11:24 A.M.
Vital Signs
Time Results Normal Range
Temperature (Temp) 39℃ 36.5℃-37.5℃
Respiratory Rate (RR) 34 breaths per minute 16-22 breaths per minute
Heart Rate 111 bpm 60-110 beats per minute
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Blood Pressure 111/63 mmHg 108/62 mmHg
SpO2 92% 95%-100%

Neonatal and Pediatric Respiratory Care, Peretta
Pediatric Glasgow Coma Scale
Result Normal Level of

Consciousness
Eye Opening 3 4 - Spontaneous Lethargic
Verbal 4 5 - Oriented,
Response Appropriate
Motor Response 5 6 - Obeys commands
PGCS Score 12 -
Moderate
General Survey
Male patient TLC, age 13, of Filipino-German ancestry was admitted to

San Pedro Hospital last July 29, 2022 because of pulmonary exacerbation
associated with cystic fibrosis. The GCS score taken upon admission was 12,
which shows that the patient was at a lethargic level of consciousness.Upon
inspection, the patient presents with digital clubbing indicating hypoxemia and
evident usage of accessory muscles for breathing which indicates increased
work of breathing. The patient also presented to be shorter for the average
height for his age and thinner as supported by his low BMI. Patient was put on
oxygen by nasal cannula and was given a small intravenous (IV) fluid bolus.
Cephalocaudal Assessment
Skin
Upon inspection, the patient's skin has increased perspiration levels.
Upon palpation, there are traces of crystallized salts forming against

the sweat glands.These presence of salt indicate the increased levels of
chloride which is significant for patients diagnosed with cystic fibrosis. The
higher level of salt in the perspiration of cystic fibrosis patients represent an
impairment in the electrolyte transport of the sweat glands (Yu & Sharma,
2021).
Head
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Upon inspection, there is no evident buildup of fluids in the head of the
patient. In addition, there are no wounds or bruises. The head is
normocephalic and symmetrical. Hair is of dark brown and is evenly
distributed to the scalp and presents as oily. The patient's head is being
supported by a towel that has both ends rolled and placed on either side.
Birthmarks and swelling are not seen.
Upon palpation, there are no obvious lumps or bulges that can be felt.
Eyes
Upon inspection, the eyes of the patient are symmetrically aligned and
anicteric however it appears to be sunken, owing to his gradual loss of weight.
However, there are no signs of bleeding in the subconjunctival and retinal
areas. Eyelashes have a gentle outward curl and are dispersed uniformly.
Pupillary reflexes are normal and pupillary diameter is at 3 mm.
Ears
Upon inspection, the external canthus of the eye aligns with a horizontal line
to join with the pinna of the ear. There are no notable lesions or discharges,
and the skin of the ear is uniform with that of the face.
Upon palpation, there are no notable swelling, tenderness, or lumps. The

pinna also recoils after it is folded.
Nose
Upon inspection, the nose of the patient is straight, symmetrical, in the

middle, and consistent in color, with some discharges that are typically
associated with a runny nose. There is also apparent nasal flaring which
indicates increased work of breathing. Nasal cannula at 2 L/min was
administered.
Upon palpation, nasal polyps are present, which might indicate sinus
infection and congestion.
Mouth
Upon inspection, the lips of the patient appear to be slightly bluish and
pale, but with no apparent lesions. There is evident enamel hypoplasia and
tooth discoloration. In addition, an anterior open bite can be observed which
can be associated with nasal and sinus obstruction (Abu-Zahra, et al., 2019).
Moreover, a shrill, coarse whistling sound can be heard, which is a clinical
manifestation known as wheezing.
Upon palpation, there are no notable inflammation and swelling around

the mouth.
Neck
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Upon inspection of the neck, there are no lesions, or bruises present.
Clavicles are still intact. The trachea is located midline and there is no jugular
vein distention. There is evident use of accessory muscles which indicates the
patient's increased work of breathing.
Upon palpation, there are no notable swelling, inflammation, or masses

noted. Normal pulse is noted.
Thorax and Lungs
Upon inspection, intercostal and subcostal retractions are evident.

There is evident use of accessory muscles which indicates the patient's
increased work of breathing. There are no apparent lesions and bruises
present.
Upon palpation, the antero-posterior diameter of the chest is 1:1.

Because of the hyperinflation developed from cystic fibrosis, the patient
presents with a barrel chest. There are no notable masses and lumps over the
chest. There is no presence of a bounding pulse when palpated.
Upon percussion, dullness can be noted. This indicates the presence

of consolidation owing to the infection brought upon by cystic fibrosis.
Upon auscultation, normal heart sound can be heard at the fourth

intercostal space in the left midclavicular line, also regarded as the maximum
impulse. There is absence of normal breath sounds in the base, first and
second intercostal space, and in the trachea. Consequently, the patient
presented with high pitched wheezing during admission. There is also
occasional popping sound, known as crackles heard over the base of the
lungs which signifies accumulated secretions.
Abdomen
Upon inspection, scars are absent.
Upon palpation, the patient reports pain. Tenderness is noted.
Upon percussion, there is hyperresonance, which indicates that the abdomen

is filled with air. This indicates presence of gas for patients with abnormal
pancreatic insufficiency due to cystic fibrosis.
Upon auscultation, there are decreased bowel sounds. This can be attributed
to the patient's malabsorption caused by the pancreatic insufficiency due to
cystic fibrosis.
Upper extremities
Upon inspection, there are no IV lines connected with the patient except for
the nasal cannula delivering oxygen to the patient. There are no notable
bruises or scars. There is notable loss of figure compared to the average size
of the patient's age, signifying loss of weight and failure to thrive.
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Upon palpation, there is no warmness or coldness that can be felt on the skin,
bounding pulse is absent, and edema is not present.
Lower extremities
Upon inspection, there are no notable scars or bruises. Digital clubbing is

present which indicates hypoxemia due to the patient's diagnosis.
B. DEVELOPMENTAL TASKS
Physical Development
Developmental Met/ Rationale

Task Unmet
Hormonal Unmet A healthy weight falls in the 5 percentile up

th
Changes to the 85 percentile. Based on the height

th
and weight of the patient, the calculated

BMI is 14.2, placing the BMI-for-age below
the 1st percentile for males aged 13 years.
The patient falls within the underweight
BMI category. (Centers for Disease Control
and Prevention, 2021) Moreover, patient
suffers from pancreatic insufficiency, which
means he does not secrete enough
enzyme for digestion, thus did not meet this
category.
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Sexual Maturation Unmet Sexual changes in boys normally begin
with scrotum and testicular enlargement,
followed by penis lengthening. Following
that, pubic hair emerges. About 2 years
after pubic hair, armpit and facial hair
emerge. A year after the testes begin to
enlarge, the growth surge normally begins.
Boys in the United States typically have
their first ejaculation between the ages of
12 and 14, around a year after penile
growth begins to accelerate. Young
adolescent boys frequently suffer minor
breast growth (gynecomastia), which
normally resolves within a few years
(Graber, 2021). The patient did not meet
this category. Based on his physical
assessment on the genitalia, his testes
have not yet descended.
Growth Spurt Unmet Growth spurt in boys occurs during mid-

adolescence, between the ages of 12 and
16 (more commonly around age 13 and a
half), and typically begins a year after the
enlargement of the testes. Boys grow
approximately 4 inches (10 cm) throughout
their peak growth year (Graber, 2021). The
patient did not meet this category. He is
considered small for his height (59 in) as
the average height for a 13 years old male
is 61.4 in (156 cm).
Nutrition
Developmental Met/Unmet Rationale
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Tasks
Adolescents require adequate nourishment

for proper growth and development. Dietary
Dietary needs choices and habits formed during youth
have a major impact on future health
(Maricopa Community Colleges, 2020).
Patients with cystic fibrosis should have a
diet rich in fruits and vegetables, whole
grains, dairy products, and protein. In
addition, individuals with CF have unique
dietary requirements to maintain health.
Depending on the individual, adolescents
with cystic fibrosis may require between
2,900 and 4,500 additional calories per day
to fuel their growth during puberty. (Johns
Hopkins All Children’s Hospital, 2022). The
patient did not meet this category because
he does not follow the requirements
needed for his condition, but only
consumes the same food as his family
Brain Development

Tasks
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Sleep Unmet Teenagers sleep, on average, between 7
and 7 hours and 15 minutes per night.
However, research indicate that the
average teenager needs exactly 9 hours
and 15 minutes of sleep (Nationwide
Children’s Hospital). The patient did not
meet this category as his sleep hours
during weekdays is only 8 hours and 30
minutes; and he can only meet this
category during weekends.
Cognitive Development

Tasks
Attention Met Selective attention (the technique of

focusing on one input while tuning out
another) and divided attention both show
improvements (the ability to pay attention
to two or more stimuli at the same time)
Patient is capable on focusing during his
class while writing notes at the same time.
Memory Met There are improvements in the working

memory and long-term memory. Long-term
memory is the enormous amount of
information retained in one's life, while
working memory is the small amount of
information that may be held in mind and
employed in the execution of cognitive
activities (Cowan, 2013). Patient is capable
in both
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Processing Speed Met Processing speed is defined as the amount
of time it takes a person to complete a
mental task. It refers to how quickly a
person can grasp and react to information,
whether visual (letters and numbers),
auditory (language), or movement-based.
In other words, processing speed is the
amount of time it takes to receive and
respond to a stimulus (CogniFit, 2022). For
adolescents, they think faster than children.
Processing speed increases dramatically
between the ages of five and middle
adolescence, peaks around the age of 15,
and does not appear to change between
late adolescence and adulthood (Maricopa
Community Colleges, 2020).
Organization Met Adolescents are more conscious of their

own mental processes and can utilize
mnemonic devices and other tactics to
efficiently think and recall information
Metacognition Met "Thinking about thinking" is metacognition.

Social cognition benefits from increasing
introspection, self-awareness, and
intellectualization. Adolescents are better at
recognizing that people don't have perfect
mental control (Maricopa Community
Colleges, 2020).
19
Hypothetical Met The ability to plan ahead, foresee future
Thinking and consequences of an action, and propose
Abstract Thinking alternate explanations for events is
hypothetical thinking. This talent makes
adolescents better debaters, as they are
able to refute the assumptions of a friend or
parent. Additionally, adolescents develop a
more complex concept of probability.
Abstract thinking is the ability to think
abstractly that allows them to comprehend
the higher-order abstract logic inherent in
puns, proverbs, metaphors, and analogies.
Enchanced proficiency enables students to
understand how language can be utilized to
convey different themes, such as satire,
metaphor, and sarcasm. In addition, this
allows advanced reasoning and logical
processes to be used to social and
ideological issues including interpersonal
relationships, politics, philosophy, religion,
morality, friendship, faith, justice, and
honesty. (Maricopa Community Colleges,
2020).
Intuitive and Met Intuitive thought is more experiential and

Analytical emotional, and it is automatic,
Thinking unconscious, and rapid. Analytic thought,
on the other hand, is purposeful,
conscious, and rational (logical). Intuitive
thinking is simpler, faster, and more
common in everyday life.
20
Risk-taking Met Most teenage injuries are related to risky
conduct such as alcohol and drug use,
reckless or distracted driving, and
unprotected sex (Maricopa Community
Colleges, 2020). Experts suggest that
adolescents overestimate their abilities
before leaving home. Recent investigations
of the neurological system demonstrate
that impulse-suppressing brain regions
don't grow until early adulthood (Graber,
2021).
Relativistic Met Adolescents are more inclined to engage in

Thinking relativistic thinking, which means they are
more likely to question the assertions of
others and are less likely to accept
information as absolute fact. They realize
outside of the familial circle that the rules
they were taught as absolute are actually
relativistic. They begin to distinguish
between norms based on common sense
and those based on culturally relative
standards. This can result in a phase of
doubting authority in all areas.
ANATOMY AND PHYSIOLOGY
With Cystic fibrosis, the organ systems that are involved include: the
respiratory system, digestive system, integumentary system, and reproductive
system. In the case of patient T.L.C., only the respiratory system, digestive
system, and reproductive system was addressed.
RESPIRATORY SYSTEM
21
UPPER RESPIRATORY TRACT
The upper respiratory tract is made up of the nose, mouth, sinuses,

pharynx, and larynx. The respiratory system is lined with a mucous
membrane that secretes mucus. Smaller particles, such as pollen or smoke,
are trapped by the mucus. Cilia, which look like hairs, line the mucous
membrane and move particles trapped in mucus out of the nose. The tissue
that lines the nasal cavity moistens, warms, and cleanses the air we breathe.
(Dugdale, III, MD, 2022)
NOSE
The nose has an external and

internal portion that is located inside the
skull. It is made up of an upper bone
framework (nasal bones, the nasal part of
the frontal bones, and the frontal
processes of the maxillae), a series of
cartilages in the lower part, and a small
zone of fibro-fatty tissue that forms the
lateral margin of the nostril (the ala). It is
divided in two by the nasal septum. The
nasal septum is usually straight at birth and remains straight throughout early
childhood, but it bends toward one side as a person age. This can cause
nasal cavity obstruction, making breathing difficult. The nasal cavity is the
interior part superior and posterior to the nose. (Taylor, 2020)
MOUTH
The mouth, also referred to as the oral

cavity, is where the respiratory system's
secondary external opening is located. Normal
breathing is primarily accomplished through the
nasal cavity, but when necessary, the oral
22
cavity can be used to support or even take the place of the nasal cavity. The
air entering the body through the mouth has a shorter pathway than the air
entering through the nose, so it does not warm and moisturize the air entering
the lungs as effectively as the air entering through the nose does. Additionally,
the nasal cavity's filtering mucus and hairs do not exist in the mouth. One
benefit of breathing through your mouth is that more air can enter your body
more quickly due to its larger diameter and shorter distance. (Taylor, 2020)
NASAL CAVITY AND SINUSES
The nasal cavity extends

from the nostrils to the choanae,
which are oval openings between
the nasal cavities and the
nasopharynx. The vestibule, a
dilated chamber inside each nostril
that is lined with coarse hairs or
vibrissae and stratified squamous
epithelium, is located in the
anterior part of the nasal cavity
inside each the bone and cartilage
nasal septum separate the nasal cavity into right and left chambers known as
nasal fossae. The conchae consist of mucous membranes supported by thin
scroll-like turbinate bones. The conchae greatly increase the surface area of
the mucus membrane over which air travel. The mucus membrane contains
mucus secreting goblet cells and an extensive network of blood vessels that
deliver heat and moisture. The hard palate forms the floor of the nasal cavity
and separates it from the oral cavity.
PHARYNX
The pharynx, commonly called the

throat, is a muscular, funnel-shaped
passageway inside the body. It connects the
23
mouth and nose to the esophagus, the pharynx is divided into 3 regions: the
nasopharynx, oropharynx, and laryngopharynx. The pharynx's superior
portion, or nasopharynx, is located in the back of the nasal cavity. The
oropharynx, which is situated at the back of the oral cavity, is where the nasal
cavity's inhaled air descends after entering the nasopharynx. At the
oropharynx, breath inhaled through the mouth travels into the pharynx.
(Dugdale, III, MD, 2022)
LARYNX
The primary function of the larynx in

humans and other vertebrates is to protect the
lower respiratory tract from aspirating food into
the trachea while breathing. It also contains the
vocal cords and functions as a voice box for
producing sounds. The larynx is located in the
anterior portion of the neck, just inferior to the
hyoid bone and superior to the trachea. The
larynx is composed of several cartilage structures
that give it structure. The epiglottis is a cartilage
piece of the larynx that serves as the larynx's
cover during swallowing. The thyroid cartilage is inferior to the epiglottis and is
often referred to as the Adam's apple because it is most commonly enlarged
and visible in adult males. The thyroid keeps the anterior end of the larynx
open and protects the vocal folds. The cricoid cartilage, which is ring-shaped
and supports the posterior end of the larynx, is inferior to the thyroid cartilage.
(Kaylie, 2022)
LOWER RESPIRATORY TRACT
The trachea, the bronchi and

bronchioles, and the alveoli, which make up the
lungs, comprise the lower respiratory system or
lower respiratory tract. These structures receive
24
oxygen from the upper respiratory system and release carbon dioxide in
exchange (Visible Body, 2022)
TRACHEA
The trachea is a hollow, tube-like structure that extends from the

larynx, or voice box, to the bronchi, which are the two passages connecting
the trachea to the lungs. It is lined with cilia, which are fine hair-like structures.
These aid in the removal of mucus containing debris or pathogens from the
trachea. The trachea's primary purpose is to transport air to and from the
lungs. In addition to delivering air, the trachea aids in disease prevention.
Before entering the lungs, the mucus in the trachea helps trap
microorganisms such as viruses and harmful bacteria (Fletcher, 2020).
BRONCHI
The bronchi are one of the two structures

(the other one is the trachea) combined to
form the tracheobronchial tree of the
lungs. The bronchi are the limbs of the
lungs' tree. The bronchi are responsible
for distributing air throughout the lungs
until it reaches the respiratory bronchioles
and alveolar sacs (these structures
pertain to the respiratory zone). The transverse thoracic plane (also known as
the sternal angle at the fourth thoracic vertebra) is where the trachea divides
into two main bronchi, one for each lung. The main bronchi (also known as
the primary bronchi) enter the lungs through the inferior and lateral hila. The
two main bronchi are not equally separated at the bifurcation. The right main
bronchus is larger in diameter, shorter, and more vertical in relation to the
hilum. To reach the left lung's hilum, the left main bronchus has a smaller
diameter and is more horizontal; it must pass inferior to the arch of the aorta
and anterior to the esophagus and thoracic aorta (Amador, Weber,&
Varacallo, 2021).
25
ALVEOLI
Alveoli are tiny air sacs in the

lungs that resemble balloons. Alveoli
serve to transport oxygen and carbon
dioxide (CO2) molecules into and out
of the bloodstream. Alveoli are an
essential component of your
respiratory system, which consists of
the organs and tissues that let you
breathe. Alveoli are the tiniest respiratory system
structures. They are placed in clusters throughout
the lungs at the end of the branches of the
respiratory tree, which is a tree-like structure of
airways that transport air into the lungs. Alveolar
walls are quite thin. This facilitates the exchange
of oxygen and carbon dioxide between the alveoli
and capillaries, which are extremely small blood
vessels (Edridge, 2022).
DIGESTIVE SYSTEM
Gastrointestinal symptoms are rather common to patients with CF. The

majority of CF patients are pancreatic insufficient, which means that the
pancreas does not secrete enough enzyme to properly digest food.
MOUTH
Food enters the digestive

tract through the mouth, or oral
cavity, which is lined by mucous
membrane. The anterior
26
entrance is protected by the lips (labia), the lateral walls by the cheeks, the
anterior roof by the hard palate, and the posterior roof by the soft palate. The
uvula is a fleshy fingerlike protrusion of the soft palate that extends inferiorly
from the soft palate's posterior border. The floor of the mouth is occupied by
the muscular tongue. The tongue has multiple bony attachments, two of which
are to the hyoid bone and the skull's styloid processes (Marieb, 2019)
PHARYNX
Food travels posteriorly from the mouth into the oropharynx and
laryngopharynx, which are frequent channels for food, fluids, and air. The
pharynx is divided into three sections: the nasopharynx, which is part of the
respiratory tract; the oropharynx, which is
posterior to the oral cavity; and the
laryngopharynx, which connects to the
esophagus below (Marieb, 2019)
ESOPHAGUS
The esophagus, or gullet, connects the pharynx to the stomach through

the use of the diaphragm. It is simply a channel that transports food to the
stomach (through peristalsis) and is about 25 cm (10 inches) in length.
27
STOMACH
The stomach, which is shaped like

a C, is located on the left side of the
abdominal cavity, almost covered by the
liver and diaphragm (Marieb, 2019). Its
function is to temporarily store food, contract and relax in order to combine
and break down food, and to produce enzymes and other specialized cells in
order to digest food (Cleveland, 2021).
SMALL INTESTINE
The small intestine is the

primary digestive organ of the body. It
digests food by using pancreatic
enzymes and bile from the liver
(Cleveland, 2021). The small intestine
is a muscular tube that connects to the
large intestine via the pyloric sphincter.
It is the longest part of a living person's
alimentary tube. The small intestine is
divided into three sections: the
duodenum, the jejunum, and the ileum (Marieb, 2019).
LARGE INTESTINE
28
The large intestine is considerably larger in diameter but is much
shorter in length than the small intestine. It reaches all the way from the
ileocecal valve to the anus. Its primary functions are to absorb water and dry
up indigestible food remnants before excreting them as feces. It surrounds the
small intestine on three sides and is divided into the cecum, appendix, colon,
rectum, and anal canal (Marieb, 2019).
ACCESSORY DIGESTIVE ORGANS
TEETH
The function of teeth is in

food processing. The teeth tear
and grind the meal, shredding it
into smaller pieces.
SALIVARY GLANDS
Three pairs of salivary

glands secrete saliva into the
mouth. The parotid glands are
located in front of the ears. The
submandibular glands and
sublingual glands secrete into the
floor of the mouth via tiny ducts.
Saliva is a mixture of mucus and
serous fluids produced by the salivary glands. Mucus moistens and helps to
bind food, making chewing and swallowing easier. The clear serous section
includes a salivary amylase enzyme, a bicarbonate-rich (alkaline) juice, that
starts the starch digesting process in the mouth.
29
PANCREAS
The
pancreas is a
soft, pink
triangle gland
that runs from
the spleen to
the
duodenum in
the abdomen
(Marieb, 2019). It secretes digestive enzymes into the duodenum, which help
to break down protein, lipids, and carbs. The pancreas also produces insulin,
which is then released into the bloodstream. Insulin is the primary hormone in
your body that regulates sugar metabolism (Cleveland, 2019).
LIVER AND GALLBLADDER
The liver is the body's biggest gland. It overlies and almost fully covers
the stomach and is located beneath the diaphragm on the right side of the
body. Bile production is its digestive functions. Bile is a yellow-to-green watery
solution that contains bile
salts, bile pigments,
cholesterol, phospholipids,
and a variety of electrolytes.
Only the bile salts and
phospholipids assist during
digestion process, while the
gallbladder is a tiny, thin-
walled green sac that nestles
in a shallow fossa on the
liver's inferior surface. When
food digestion is not taking place, bile accumulates in the cystic duct and
enters the gallbladder to be stored (Marieb, 2019).
30
REPRODUCTIVE SYSTEM
TESTES
Men often have two testicles. The

spermatic cord secures the testes at either end,
which are oval organs the size of very big olives
that are located in the scrotum. The production of
sperm and testosterone, the main hormone used
in male sex, is carried out by the testes.
DUCT SYSTEM
EPIDIDYMIS
There are four anatomical parts in

the epididymis: the initial segment, caput,
corpus, and cauda. The epididymis is a
duct-like structure that connects the testis to
the vas deferens. The interaction of sperm
cells with the distinct luminal environment of
each epididymal area causes sperm
maturation to take place during epididymal
transit. The epididymis as a key
reproductive organ in charge of sperm
31
maturation, storage, preservation, and
concentration. (James ER, 2020)
DUCTUS DEFERENS
The ductus deferens, or sperm duct, is

another name for the vas deferens. This lengthy
muscular tube connects to your urethra via the
ejaculatory duct and travels from the epididymis
into the pelvic cavity behind your bladder. Your
spermatic cord surrounds your vas deferens, which
moves mature sperm to the urethra before to
ejaculation.
URETHRA
Your urinary system includes the

urethra. Your kidneys, bladder, ureters, and
urethra are all parts of this system. Your
kidneys cleanse your blood and create
urine, a waste product. Two ureters
transport the urine from your two kidneys to
your bladder, where it is held until you need
to urinate. The hole at the end of your
urethra is where urine exits the body.
ACCESSORY GLANS AND SEMEN
32
SEMINAL VESICLE
The male pelvis has a pair of

glands called the seminal vesicles, often
referred to as the vesicular or seminal
gland, which are responsible for producing
many of the chemicals that make up
semen. In the end, they contribute roughly
70% of the entire amount of semen. The
vas deferens, which joins with the duct of
the seminal vesicles to produce the
ejaculatory duct and then empties into the prostatic urethra, is their most
significant anatomical connection.
PROSTATE
The prostate serves a number of purposes.

The production of seminal fluid, a part of semen, is
the most significant. Additionally, it affects hormone
synthesis and controls urine flow. In particular, older
men frequently experience prostate issues. Prostate
cancer, enlargement of the prostate, and
inflammation of the prostate are the most frequent.
(Newman, 2021)
BULBO-URETHRAL GLANDS
A pair of pea-shaped exocrine glands called the

bulbourethral glands, also referred to as Cowper's
glands, are positioned posterolateral to the
membranous urethra. By secreting a lubricating mucus,
they add to the ultimate amount of semen. The gland's
ducts enter the proximal region of the spongy urethra
through the perineal membrane, which is located next
to the membranous urethra.
33
SEMEN
During an orgasm, a man's penis releases this chemical. The prostate

glands and seminal vesicles release a white fluid known as seminal fluid,
which contains the sperm. Combined with sperm, this fluid is known as
semen. It contains: Sperm, male genitalia cells. The distinctively shaped cells
contain: Head, which contains DNA for fertilizing an egg in a woman; Tail that
facilitates the fertilization of the egg by allowing it to "swim" through the
female reproductive system; and Fluids, which allow sperm to be placed
toward the woman's vagina's rear. This region lies near the cervix, which is
where the womb's opening is located and where babies develop.
PATHOPHYSIOLOGY
Cystic fibrosis is an exocrine gland disease that affects multiple organ

systems but primarily causes chronic respiratory infections, pancreatic
enzyme insufficiency, and other complications in untreated patients.
A. Factors
PREDISPOSING FACTORS
FACTOR PRESENT/ABSENT RATIONALE
Family Present Family history of cystic fibrosis is the most

History important risk factor, especially if either
parent is a known carrier. Cystic fibrosis is
caused by a recessive gene. This means
that offspring must inherit two copies of the
gene, one from each parent, in order to
have cystic fibrosis. If a child only receives
one copy of the gene, he or she will not
develop cystic fibrosis. That child, however,
will remain a carrier and may pass the gene
on to his or her own children.
Secondhand Present Secondhand smoke increases the chance

smoke of infections and other problems almost as
34
much as smoking. Research from the Johns
Hopkins University School of Medicine
shows that secondhand smoke causes the
capacity and function of the lungs to go
down by 10%.(How Genetics and Lifestyle
Contribute to Cystic Fibrosis Risk, 2021)
Ethnicity Most common metabolic disease that lasts

for a long time in Caucasian European
children.
Gender Present Males have slightly more than half of all

cases of cystic fibrosis (CF), but until about
age 20, they usually do better than females.
After that, the chances of long-term survival
are about the same for men and women.
(Pool, 2019)
Pulmonary Present Pseudomonas aeruginosa (PsA) is a type of

Infection bacteria that is often found in the lungs of
people with cystic fibrosis (CF); the patient
has this in his past health history and a
long-term infection with PsA is linked to a
steady decline in pulmonary function. CF
care providers have a hard time getting rid
of this organism and treating it in CF
patients' airways.
PRECIPITATING FACTORS
FACTOR PRESENT/ Rationale

ABSENT
Financial Present Financial constrictions cause significant

Constrictions financial hardship as people struggle to
budget while looking after their health. It
is a burden in an average family.
B. Diagram
https://www.canva.com/design/DAFIc7xX-B8/
czWpSVYmU9SOW8RINOkwFw/edit?utm_content=DAFIc7xX-
B8&utm_campaign=designshare&utm_medium=link2&utm_source=shar
ebutton
35
C. Narrative
Cystic fibrosis is a disorder caused by mutation in the cystic fibrosis

transmembrane conductance regulatory gene known as CFTR for short this
mutation occurs on chromosome 7 this disease is an autosomal recessive
disorder which means that the disease is inherited when a child inherits a
defective copy of the gene from both parents were carriers of the gene. A
carrier is an individual who has a defective copy and a normal copy of a gene
and is unaffected themselves. The CFTR gene codes for the seat of TR
protein which is an important chloride channel, the purpose of this channel is
to pump ions in and out of the cell. The symptoms and associated
complications of cystic fibrosis are caused by the dysfunctional CFTR protein
channel which is found throughout the gastrointestinal tract, affected
individuals have elevated sweat electrolyte levels; pancreatic insufficiencies
and lung infections, these signs are observed in most patients but not all since
severity of the disease can range widely amongst individuals. In addition, in
the mid 1900s the median survival age of individuals with this disease was
eight years but today the median survival age has drastically improved to 30
years mainly due to better understanding of the disease and improved clinical
approaches.
This inherited disease mainly affects the lungs, pancreas, liver,

intestines, and sinus is the most prominent organ affected are the lungs.
Excess mucus causes infections and eventual permanent lung damage with
trapped bacteria. Normal digestive functions are also altered by this disease
as the mucus blocks digestive enzymes from reaching the intestines.
Therefore, food, specifically fats and proteins are not digested properly in
patients. Finally, inflammation and infection of the sinus is a common effect of
this disease as this disease affects the layer of epithelium found in the upper
respiratory tract.
DIAGNOSTICS
Laboratory Tests
Most Common:
Sweat (chloride) test
The gold standard for determining cystic fibrosis diagnosis is the sweat
chloride test. Thistest measures the amount of chloride found in sweat. There
are no needles involved in this test. In the first part of the test, a colorless,
odorless chemical called pilocarpine and a little electrical stimulation is
applied to a small area of the arm or leg to make the sweat glands to produce
36
sweat. Afterwards, the patient may feel warmth or a tingling sensation.. This
part of the test lasts about five minutes.
The sweat is then collected on a piece of filter paper or gauze or in a plastic

coil. This step lasts for 30 minutes. The collected sweat is then sent to a
hospital laboratory to measure how much chloride is in the sweat — usually
later the same day. The sweat test usually takes about an hour, but it may
take longer. When you schedule the test, ask how long it will take and when
you can expect to learn the results.
To understand what the sweat test results mean, a chloride level of:
Less than or equal to 29 mmol/L = CF is unlikely regardless of age*.
Between 30 - 59 mmol/L = CF is possible and additional testing is needed.
Greater than or equal to 60 mmol/L = CF is likely.
*Although a sweat test result of less than 29 mmol/L makes CF unlikely, there
are CFTR mutations associated with sweat test results of less than 29
mmol/L.
Genetic tests
Because cystic fibrosis is an autosomal recessive condition, both parents

must pass on the CFTR mutation for a child to get the condition. If you have
just one mutation, you won't get CF but you'll be able to pass it on to your
offspring as a carrier. There is no single test that can identify every variant of
37
the numerous CTFR mutations. The 23 most prevalent CFTR mutations are
intended to be detected by the common genetic test.
Blood samples are typically used for genetic testing for CF, although a swab
of cells from the inside of your cheek may potentially be used. Results are
typically available in three to five days. A positive test result indicates that you
have cystic fibrosis and two copies of the CFTR mutation, if the test is being
used to diagnose CF.The test may also be used to estimate the severity of the
condition based on the sorts of mutations you have. For instance, of the more
than 2,000 CTFR mutations, the deltaf508 mutation will be discovered in
roughly 70% of cases7. If you inherit two of these from your parents, you will
probably have a greater respiratory obstruction, a thicker mucus discharge,
and a worse functioning pancreas.
Other Tests:
Chest X-rays
This exam uses a small dose of ionizing radiation to produce images to

evaluate for dilated airways filled with mucus and to evaluate lung infections
that need to be treated with antibiotics. Doctors use chest x-rays periodically
to observe changes in cystic fibrosis patients and rule out other respiratory
conditions such as pneumonia or a collapsed lung.
Normal chest radiographic findings may be seen in infancy and even in older
children with mild pulmonary involvement, though this is uncommon. Some
study results support the lack of a precise correlation between the
radiographic picture and the clinical manifestations of exacerbation, with a
reported sensitivity and specificity of 56% and 78%, respectively. Most
patients with CF demonstrate some of the classic chest radiographic findings
that reflect chronic bronchiectasis: Hyperinflation, bronchial thickening and
dilatation, peribronchial cuffing, mucoid impaction, cystic radiolucencies,
Increase in interstitial marking, scattered nodular densities and abdominal x-
ray may show dilated loops of bowel.
DATE:
38
Chest X-ray
Arterial Blood Gas
An arterial blood gas (ABG) tests explicitly blood taken from an artery. ABG
analysis assesses a patient's partial pressure of oxygen (PaO2) and carbon
dioxide (PaCO2). PaO2 provides information about the oxygenation status
and PaCO2 provides information on the ventilation status (chronic or acute
respiratory failure). PaCO2 is affected by hyperventilation (rapid or deep
breathing), hypoventilation (slow or shallow breathing), and acid-base status.
Date: July 29, 2022 , 1:00 P.M.
Arterial Blood Gas Sampling Values
ABG Element Result Normal Ranges
pH 7.49 7.38-7.42
PaCO2 52 mmHg 38-42 mmHg
HCO3 30 mEq/L 22-28 mEq/L
PaO2 62 mmHg 75-100 mmHg
Date: August 1, 2022
pH 7.48 7.38-7.42
PaCO2 43mmHg 38-42 mmHg
39
HCO3 29mEq/L 22-28 mEq/L
PaO2 70mmHg 75-100 mmHg
DATE: August 4, 2022
pH 7.46 7.38-7.42
Interpretation:
CBC
Date: July 29, 2022, 12:05 P.M.
Complete Blood Count
Parameters Result Reference Range Interpretation
Hemoglobin (g/dL) 9.4 11.4-15.4 ↓
Hematocrit (%) 29 34-45 ↓
40
RBC Count (x 10⁶ /µL) 3.20 3.80-5.20 ↓
MCV (fL) 93 78-89 N
MCH (pg) 29 27-31 N
MCHC (%) 33 32-36 N
RDW (%) 12.5 11.4-13.5 N
WBC count (x 10³ /µL) 14.8 4.5-12.5 ↑
Differential Count
Neutrophils (%) 87 40-70 ↑
Lymphocytes (%) 15 28-48 ↓
Monocytes (%) 1.0 0-0.8 ↑
Eosinophils (x 10⁹ /µL) 0.28 0.10-0.20 ↑
Basophils (x 10⁹ /µL) 0.18 0.00-0.10 ↑
Platelet count (x 10⁹ /µL) 349 139-320 ↑
MPV (fL) 10.2 9-12 N
41
Hemoglobin (g/dL) 10.4 11.4-15.4 ↓
RBC Count (x 10⁶ /µL) 4 3.80-5.20 N
MCV (fL) 88 78-89 N
MCH (pg) 30 27-31 N
MCHC (%) 31 32-36 ↓
RDW (%) 12.4 11.4-13.5 N
WBC count (x 10³ /µL) 13.6 4.5-12.5 ↑
DATE: August 4, 2022 (7:09 AM)
Hemoglobin (g/dL) 11 11.4-15.4 ↓
Hematocrit (%) 40 34-45 ↑
RBC Count (x 10⁶ /µL) 4.90 3.80-5.20 N
MCV (fL) 93 78-89 N
MCH (pg) 29 27-31 N
MCHC (%) 34 32-36 N
42
RDW (%) 12.7 11.4-13.5 N
WBC count (x 10³ /µL) 12.8 4.5-12.5 ↑
Differential Count
Lymphocytes (%) 45 28-48 N
Monocytes (%) 0.8 0-0.8 N
Eosinophils (x 10⁹ /µL) 0.22 0.10-0.20 ↑
Basophils (x 10⁹ /µL) 0.09 0.00-0.10 ↑
Platelet count (x 10⁹ /µL) 320 139-320 N
MPV (fL) 10.3 9-12 N
Sputum culture
A sputum (mucus) CF respiratory screen or culture can help doctors detect

and identify these bacteria or fungi so they can reccomend the most effective
antibiotics to target a specific infection. A sputum culture can help identify
specific causes of infections in the lungs and airways. Such infections can
lead to coughing that produces yellow, greenish, or blood-tinged sputum, in
addition to fever and difficulty breathing. The best time for testing is usually in
the morning, before your child has had anything to eat or drink.
Date: July 29, 2022 (12:10 PM)
Sputum Culture
43
Result
Phlegm Blood-tinged or green sputum
Positiv Highly mobile, opportunistic

e
gram-negative bacillus
Pseudomonas aeruginosa
To begin the procedure, your child will be asked to rinse his or her mouth out
with water, then breathe deeply and cough deeply to produce sputum from the
airway. The child may need to tap gently on your child's chest to help loosen
the sputum in the lungs. If your child can't produce a sample, the respiratory
therapist may need to use a tongue depressor to stimulate a cough, or your
child may need to inhale a mist solution to help produce a cough.
DRUG STUDY 1
44
Date Given:
Brand Name:
Elixophylline
Pulmophylline
Quiberon-T
Theo-24
Theolair
Uniphyl
Generic Name: Theophylline
Classification: Methylxanthines
Mode of Action:
Theophylline is used to relax the smooth muscle of the pulmonary blood

vessels and bronchial airways and reduces airway responsiveness to
methacholine, histamine, adenosine, and allergen. This competitively inhibits
type III and IV PDE (phosphodiesterase), this is the enzyme responsible for
the breakdown of cyclic AMP in smooth muscle cells, resulting in
bronchodilation.
Theophylline also binds to the adenosine A2B receptor and blocks adenosine-
mediated bronchoconstriction. In inflammatory states, theophylline activates
45
histone deacetylase to prevent the transcription of inflammatory genes that
require the acetylation of histones for transcription to begin. [2]
Indication/route/dosage:
· Oral route: 300mg per day, divided and given every 6 to 8 hours. [4]
Contraindications:
Contraindicated in patients with a history of hypersensitivity to theophylline or

other components of the product.
Side effects:
Chest pain or discomfort, dizziness, fast, slow, or irregular heartbeat, increase

in urine volume, and lightheadedness.
Adverse effects:
Persistent vomiting, pounding or rapid pulse, and seizures.
Drug interaction: [3]
Taking theophylline and PDE inhibitors (riociguat) blood pressure may

decrease causing fatigue, weakness, and loss of consciousness. A heart
attack or stroke is also possible.
When Theophylline and Xanthine Derivatives are taken together, the

effectiveness of dipyridamole might be less specifically on the blood vessels.
Theophylline and Carbamazepine, when taken together the body will process
one or both medicines more quickly.
46
Combining theophylline and albuterol may intensify the risk of cardiovascular
side effects including palpitations, accelerated heart and pulse rates, and
blood pressure increases. Taking these drugs together may increase the
chance of getting hypokalemia, or low blood potassium.
RT Management:
Assess patient history.

Rationale: This is to make sure that the patient/s is not sensitive to any
xanthines
Asses patients’ physical appearance before and after administering the drug.
This includes skin texture, color, lesions, as well as reflexes, vital signs, and
breath sounds.
Rationale: Adverse effects may occur after drug is being administered
Inform the patient to take the medication as instructed.

Rationale:Overdosing of medication may lead to serious health problems
Inform patient to avoid excessive intake of tea, coffee, chocolate or any

theophylline-related substances. This will increase the side effects of the
medication.
Rationale: This will increase the side effect of the medication
47
Monitor results of serum theophylline level determinations carefully, and
reduce dosage if serum levels exceed therapeutic range of 10–15 mcg/mL.
Rationale: Heart attack may occur if amounts of theophylline are excessive
than usual.
Monitor and assess for clinical signs of side and adverse effects, specifically if
theophylline levels are not available.
Rationale: By assessing the clinical signs of adverse effects, further
exacerbation of the undesirable effects can be lessened.
Inform patient to seek professional help when experiencing adverse effects.

Rationale: To make sure patient gets immediate treatment response.
DRUG STUDY 2
Date Given:
48
Brand Name: Ultrase MT
Generic Name: Pancrelipase
Drug Class: Pancreatic/Digestive Enzymes
Mode of Action:
Pancrelipase is a type of prescription medication that can be used to treat

partial or complete exocrine pancreatic insufficiency caused by cystic fibrosis,
chronic pancreatitis, pancreatectomy, pancreatic obstruction, and other
pancreatic diseases.
Pancreatic medications are made to avoid inactivation by gastric acid which

results in the delivery of high levels of biologically active enzymes into the
duodenum. Then the enzymes will catalyze the hydrolysis of fats into glycerol
and fatty acids, starch into dextrins and sugars, and protein into proteoses
and derived substances.
Dosage:
In general, daily requirements of 500-4000 lipase units/g of fat are required.
Not to exceed 10,000 unit lipase/kg/day or less than 4,000 lipase unites/g fat
ingested daily.
Contraindications:
Documented hypersensitivity.
Side Effects:
Some common side effects are: abdominal pain/cramping, bloating,

headache, weight loss, flatulence, nausea, vomiting, dyspepsia, cough,
diarrhea, and constipation.
49
Adverse Effect:
The undesirable side effects of this drug are: fibrosing colonopathy,

hyperuricemia, anal pruritus, biliary tract stones, and allergic reactions.
RT Management:
Assess the patient's medical history. Check if the patient is allergic to the
medication.
Rationale: To know what are the past medications of the patient.
breath sounds.
Rationale: To obtain baseline data of the patient.
Assist the patient when needed to take the medication. Swallow pills as
chewing or retaining in the mouth may cause oral mucosa irritation; do not
chew, crush or mix in foods having a pH above 4.5
Rationale: This is to ensure that the patient is taking the medication and
proper preparation of the drug is followed.
Avoid exceeding maximum dose due to risk of fibrosing colonopathy; more

than or equal to 6,000 lipase units/kg per meal associated with colonic
stricture, indicative of fibrosing colonopathy, particularly in children below 12
years
Rationale: To avoid drug overdose
50
Monitor uric acid levels in patients with gout, hyperuricemia, or renal
impairment due to risk of hyperuricemia.
Rationale: this is important since one of the adverse effects is hyperuricemia.
Taking the drug as prescribed will ensure the effectiveness of the drug.
Inform patient to seek help when experiencing side and adverse effects.
Rationale: To facilitate them effectively.
DRUG STUDY 3
Date Given:
Brand Name: Pulmozyme
Generic Name: Dornase alfa
Classification: Mucolytic
Mode of action:
Pulmozyme (dornase alfa) is a mucolytic that, in preclinical laboratory studies,

was shown to target and split extracellular DNA to decrease mucus viscosity.
51
It is a solution of recombinant human deoxyribonuclease I, which is an
enzyme that has a primary amino acid sequence that is identical to the native
human enzyme. Pulmozyme is inhaled orally, it directly goes to the lungs to
help reduce viscosity by cleaving extracellular DNA. This breaks up sticky
mucus, making it looser and thinner.
Indication/Route/Dosage: [5]
Cystic Fibrosis
2.5 mg via oral inhalation using a nebulizer taken twice daily.
Contraindication:
Pulmozyme is contraindicated in patients who demonstrated hypersensitivity

to hamster protein.
Side effects:
Rash, sneezing, and stuffy nose
Adverse effects:
The moderate adverse effect of this drug includes chest pain, dyspnea, and
conjunctivitis. Moderate adverse effects include pharyngitis, headache,
rhinitis, fever, hoarseness, laryngitis, and dyspepsia.
Drug Interaction:
There are no drug interactions associated with Dornase Alfa/Pulmozyme

products.
RT Management:
52
Perform physical examination to establish data for assessing effectiveness of
Pulmozyme and other adverse effects associated with the specific drug.
Rationale: This is to make sure the patient’s adverse effects are all expected
reactions
Assess for possible cautions and contraindications. This includes any px

history of allergies to drugs, asthma, and persistent cough due to smoking.
Rationale:This is to make sure the patient is physically capable and prepared
for medication.
Assess skin for any presence of color and lesions to monitor any adverse
effects.
Rationale: Any significant changes in skin discoloration or lesions must be
noted
Assess respirations and breath sounds to evaluate the response of the

respiratory to the drug.
Rationale: The drug eases the work of breathing, any negative changes in
respiration must be noted.
Monitor pulse and blood pressure to evaluate cardiac response to

Pulmozyme.
Rationale:Blood pressure values and cardiac function might be affected by the
drug therefore must be noted.
53
Monitor patients’ temperature to assess underlying infections.
Rationale: This is to make sure infection is not present.
Inform patient to seek professional help when experiencing adverse effects.
Rationale: This encourages the patient to seek help when necessary.
DRUG STUDY 4
Date Given:
Brand Name: TOBI Podhaler
Generic Name: tobramycin inhalation powder
Classification: Aminoglycosides
Mode of action:
TOBI Podhaler consists of a dry powder formulation of tobramycin for oral

inhalation only with the Podhaler device. The inhalation powder is filled into
clear, colorless hypromellose capsules.
54
Each clear, colorless hypromellose capsule contains a spray dried powder of
28 mg of tobramycin active ingredient with 1,2-distearoyl-sn-glycero-3-
phosphocholine (DSPC), calcium chloride, and sulfuric acid (for pH
adjustment).
Indication/Route/Dosage:
The capsules should not be swallowed as the intended effects in the lungs will
not be obtained. Therefore these capsules are only for oral inhalation and
should only be used with the Podhaler device. The recommended dosage of
TOBI Podhaler for both adults and pediatric patients 6 years of age and older
is the inhalation of the contents of four 28 mg TOBI Podhaler capsules twice-
daily for 28 days using the Podhaler device. Dosage isn’t adjusted by weight.
Each dose of four capsules should be taken as close to 12 hours apart as
possible and each dose shouldn’t be taken less than 6 hours apart.
Contraindication:
The drug is contraindicated in patients with a known hypersensitivity to any

aminoglycoside.
Side effects:
Cough, sore throat, hoarse voice,shortness of breath, noisy

breathing,worsening of lung problems or cystic fibrosis, coughing up mucus or
blood, altered sense of taste, fever, headache, and rash.
Adverse effects:
Hives, difficulty breathing, swelling of your face, lips, tongue, or throat,

wheezing, chest tightness, trouble breathing, hearing problems, ringing in
your ears, severe dizziness, spinning sensation, balance problems, weak or
shallow breathing, muscle weakness, little or no urination, swelling in your feet
or ankles, and fatigue.
Drug Interaction:
No clinical drug interaction studies have been performed with TOBI Podhaler.
In clinical studies, patients receiving TOBI Podhaler had continued to take
other drugs like dornase alfa, bronchodilators, inhaled corticosteroids, and
55
macrolides. No clinical signs of drug interactions with these medicines were
identified. Concurrent and/or sequential use of TOBI Podhaler with other
drugs with neurotoxic, nephrotoxic, or ototoxic potential should be avoided.
Some diuretics can enhance aminoglycoside toxicity by altering antibiotic

concentrations in serum and tissue. TOBI Podhaler should not be
administered concomitantly with ethacrynic acid, furosemide, urea, or
intravenous mannitol. The interaction between inhaled mannitol and TOBI
Podhaler has not been evaluated.
RT Management:
Conduct a physical examination and medical history to establish data for

assessing effectiveness of the drug and other adverse effects associated with
the specific drug.
Rationale:: To know the past medical history of the patient and to determine
whether or not the treatment will be effective for the patient.
Assess for possible cautions and contraindications. This includes any history
of allergies to drugs, asthma, and persistent cough.
Rationale: To obtain baseline data from the patient.
effects.
Rationale: To treat the effects immediately if symptoms or any signs of any
adverse effects are taking place.
56
Assist the patient when taking the drug in case of accidentally swallowing the
drugs as this drug is intended for Inhalation.
Rationale: This drug is intended for inhalation use and the effects of the
medication may not be effective.

Rationale: Monitoring the respiratory and breath sounds will help the RT to
reduce the side and adverse effects which may worsen.
Monitor pulse and blood pressure to evaluate cardiac response.

Rationale: High blood pressure and faster pulse may indicate effects of the
drug that may not be the intended result.
Inform patient to seek professional help when experiencing undesirable side

effects as it may escalate into serious problems.
Rationale: As it may escalate into serious health problems.
57
DRUG STUDY 5
Date Given:
Brand Name: Claritin
Generic Name: loratadine
Classification:
Antihistamines
2nd Generation
Antihistamine/Decongestant Combos
Mode of action:
Claritin is a prescription medicine which is classified as antihistamines used to

relieve the symptoms of hay fever or other upper respiratory allergies. Claritin
may be used alone or with other medications.
The drug is meant for oral intake.
adults and children 6 years and over: not more than 1 tablet in 24 hours
children under 6 years of age: ask a doctor
58
Contraindication:
Patients with documented hypersensitivity to the drug or components of the

formulation should not take the drug.
Side effects: The most common side effects of this drug are: Headache,
feeling tired or drowsy, stomach pain, vomiting, dry mouth and feeling nervous
or hyperactive
Adverse effects:
This drug may cause serious side effects like: fast or uneven heart rate,
severe headache and feeling lightheaded
Drug Interaction:
Interactions may occur with certain antibiotics, antifungal medications, and

acid-reducing drugs. Precaution may apply to individuals who have asthma,
kidney disease, or liver disease. People who have phenylketonuria (PKU)
should avoid certain brands of orally disintegrating tablets that may contain
aspartame.
RT Management:
Assess the patient's medical history. Check if the patient is allergic to any
ingredients from the medication.
Rationale: To know the past medical history of the patient and to determine
whether or not the patient is allergic to any component of the drug.
breath sounds.
Rationale: To treat the effects immediately if the symptoms and any signs of
adverse effects are taking place.
59
Assist the patient when needed to take the medication. Swallow pills as
chewing or retaining in the mouth may cause oral mucosa irritation; do not
chew, crush or mix in foods having a pH above 4.5
Rationale: The pills are meant to be swallowed. Chewing or retaining in the
mouth may cause oral mucosa irritation.
Ask a doctor before use if you have liver or kidney disease. Your doctor
should determine if you need a different dose.
Rationale: Taking more than directed may cause drowsiness.
Inform the patient to seek help when experiencing side and adverse effects or
any sudden allergic reactions.
Rationale: To give immediate attention to the symptoms of the side and
adverse effects and allergy symptoms.
DRUG STUDY 6
Date Given:
60
Brand Name: Kefzol
Generic Name: Cefazolin
Classification: Cephalosporins
Mode of Action: [6]
Cefazolin works by interfering with bacterial cell wall synthesis. This cell wall
contains peptidoglycan which works to keep particles and other external fluids
from entering the cell. By disrupting that synthesis, the antibiotic blocks the
protein that links peptidoglycans, leaving bacterial cell walls open. Cefazolin
binds to 1 or more of the penicillin-binding proteins which then inhibits the final
transpeptidation step of peptidoglycan synthesis in the bacterial cell wall.
Then arresting cell wall assembly and inhibiting biosynthesis which results in
bacterial cell death.
Cystic Fibrosis
IV or IM: 25 to 50 mg/kg/day, 3 divided doses.
Contraindication:
Contraindicated in patients who have a history of immediate hypersensitivity

reactions to cephalosporin class of drugs.
Side effects:
Bleeding, blistering, burning, coldness, discoloration of the skin, feeling of

pressure, hives, infection, and inflammation.
Adverse effects:
61
Seizures, Urticaria, diarrhea, Stevens-Johnson syndrome, erythema
multiforme, and toxic epidermal necrolysis
Drug Interaction:
Cefazolin may decrease the excretion rate of Abacavir which could result in a
higher serum level.
The excretion of Acamprosate can be decreased when combined with

Cefazolin.
The risk or severity of nephrotoxicity can be increased when Cefazolin is

combined with Aceclofenac.
The therapeutic efficacy of Abciximab can be decreased when used in

combination with Cefazolin.
The risk or severity of bleeding can be increased when Cefazolin is combined

with Acenocoumarol.
RT Management:
62
Determine any previous allergic reactions toward penicillin or any antibiotics.
Rationale: To provide immediate treatment for allergies when it occurs.
Monitor for changes in intake and output (I&O) ratio, creatinine levels and
possible kidney damage.
Rationale: To provide immediate treatment and to stop the medication if
necessary.
Report diarrhea as an onset of life-threatening Pseudomembranous colitis.

Rationale: Immediate response should be given if diarrhea is reported.
Monitor for seizures.

Rationale: To determine when to stop administrating the medication and to
provide immediate treatment for this event.
Immediately notify the physician if the patient increases or develops seizure

activity.
Rationale: To stop administrating the medication.
Monitor signs of pseudomembranous colitis, this includes abdominal pain,

diarrhea, presence of mucus in stools, fever, and severe gastrointestinal
problems.
63
Rationale: To determine whether or not to continue the treatment with this
drug.
DRUG STUDY 7
Date Given:
Brand Name:
Cipro
Proquin XR
Generic Name: Ciprofloxacin
Classification: Antibacterial, Fluoroquinolone
Mode of Action:
Ciprofloxacin acts on bacterial topoisomerase IV and topoisomerase II (DNA

gyrase). Ciprofloxacin's targeting of the alpha subunits of DNA gyrase
prevents it from supercoiling the bacterial DNA which prevents DNA
replication.
Treatment of infections caused by susceptible gram-negative bacteria,

including Pseudomonas aeruginosa.
64
Taken orally with a dosage of 20 ml/kg in a 2-repeat dose taken every 12
hours
Contraindication:
Hypersensitivity
Should not be used with tizanidine
Avoid strong sunlight or sun lamp exposure during treatment.
Side Effects:
Stomach pain, headache, vomiting, nausea, diarrhea, blurring of vision
Adverse Effects:
damage to your nerves (which may be permanent), tendon problems,

behavior changes (after just one dose), or low blood sugar which can lead to
coma.
Drug Interaction:
Taking any of the following drugs at the same time can make ciprofloxacin
less effective.
didanosine (Videx) powder or chewable table
65
Ulcer medicines (sucralfate, antacids)
Clozapine, theophylline, cyclosporine, phenytoin
NSAIDs (aspirin, ibuprofen, naproxen)
Take cipro dose 2 hours before or 6 hours after taking the other medicines.
RT Management:
Assess patient history: allergy to ciprofloxacin or other quinolones, seizures,

renal dysfunction.
Rationale: This is to make sure the medication is safe for the patient.
Monitor for skin color, orientation, mucus membranes, bowel sounds, reflexes,
and lesions.
Rationale:
Inform patient to take plenty of fluids while taking the drug (well hydrated)
Rationale: Hydration is important in all aspects of medicine.
66
Encourage the patient to complete a full course of therapy
Rationale: This is to allow the patient to enjoy the entire benefit of the drug.
Monitor clinical response, and take note of the improvements.

Rationale: This is to note the significant improvement after administration of
drug.
Ensure that patient swallows tablets whole. (do not crush, cut, or chew)
Rationale: This is to make sure the patient is informed that the tablet should
be swallowed whole.
Rationale: This is to encourage the patient to not tolerate any adverse effects
that bring discomfort.
DRUG STUDY 8
67
Date Given: July 29, 2022 (12:20 PM)
Brand Name:
Beclovent
Vanceril
Vanceril DS
Qvar with Dose Counter
Generic Name: Beclomethasone
Classification: Inhaled corticosteroids
Mode of Action:
Beclomethasone dipropionate is a corticosteroid and prodrug that is rapidly

activated by hydrolysis to the active monoester, 17 monopropionate (17-
BMP), which mediates anti-inflammatory actions. 17-BMP has been shown in
vitro to exhibit a binding affinity for the human glucocorticoid receptor which is
approximately 13 times that of dexamethasone and 25 times that of
beclomethasone dipropionate. Upon binding of the ligand, the glucocorticoid
receptors dimerize and translocate into the nucleus, where they subsequently
bind to glucocorticoid response elements (GRE) on glucocorticoid-responsive
genes, leading to changes in transcription. There are several proposed
mechanisms for the anti-inflammatory action of corticosteroids.
Corticosteroids may work by increasing the transcription of genes coding for
anti-inflammatory proteins, including lipocortin-1 and interleukin-10.
Corticosteroids were also shown to inhibit the expression of multiple genes

that encode pro-inflammatory factors, such as cytokines, chemokines, and
adhesion molecules, that are activated during the chronic inflammatory
process This is thought to be due to the direct inhibitory interaction between
activated glucocorticoid receptors and activated pro-inflammatory transcription
factors, such as nuclear factor-kappa B and activator protein-1.
68
For patients receiving inhaled corticosteroids
Oral Inhalation: 50-100mcg inhaled twice daily. Maximum dose: 400mcg

taken twice daily.
Contraindications:
Use with caution specifically in respiratory infections. Beclomethasone can

cause paradoxical bronchospasm occasionally.
Hypersensitivity. Acute infections uncontrolled by antimicrobial chemotherapy.
Side effects:
Yeast infection particularly in patients’ mouth, sinus pain, cold symptoms

(stuffy nose, sore throat, sneezing)
Adverse effects:
Angioedema, bronchospasm, rash, urticaria, systemic adrenal suppression,

and oral candidiasis.
Drug interaction:
Inhaled corticosteroids are substrates of CYP3A4 (hepatic) and have the

potential to interact with ritonavir. These interactions would result in adrenal
suppression, Cushing’s syndrome, and steroid accumulation.
RT Management:
69
Assess patient history including acute asthmatic attack, status asthmaticus;
systemic fungal infections; allergy to any ingredient; lactation; untreated local
infections, nasal septal ulcers, recurrent epistaxis, nasal surgery, or trauma.
Rationale: This is a great help for the doctors to make a diagnosis for the
patient.
Taper systemic steroids carefully during transfer to inhalational steroids.

Rationale: To avoid death resulting from adrenal insufficiency during and after
transfer from systemic to aerosol steroids.
Inform patient that respiratory inhalant has been prescribed to prevent

asthmatic attacks, not for use during an attack.
Rationale: To avoid drug overdose.
Allow 1 minute between puffs

Rationale: This gives the medicine and propellant enough time to mix.
Rinse your mouth after using the respiratory inhalant aerosol.

Rationale: To avoid oral candidiasis.
70
Use a decongestant before the intranasal steroid, and clear your nose of all
secretions if nasal passages are blocked; intranasal steroids may take several
days to produce a full benefit.
Rationale: This is to reduce nasal swelling
Inform patient to take the medicine exactly as prescribed; do not take more
than prescribed, and do not stop taking the drug without consulting your
health care provider.
Rationale: To avoid other complications.
Inform patient to seek for medical help if experiencing a sore throat or sour
mouth.
Rationale: To prevent worsening of the problem.
DRUG STUDY 9
71
Date Given:
Brand Name: Rhinocort Aqua
Generic Name: Budesonide
Classification: Corticosteroids, Intranasal
Mode of action:
Rhinocort Aqua Nasal Spray is an unscented, metered-dose, manual-pump

spray formulation that contains a micronized suspension of budesonide in an
aqueous medium. It also contains microcrystalline cellulose and
carboxymethyl cellulose sodium, dextrose anhydrous, polysorbate 80,
disodium edetate, potassium sorbate, and purified water and hydrochloric acid
is added to adjust the pH to a target of 4.5.
It delivers 32 mcg of budesonide per spray and it contains 120 metered

sprays after initial priming.
Before using the first time, the container must be shaken gently and the pump
must be primed by actuating eight times. If used daily, the pump does not
need to be reprimed. Reprime with one spray or until a fine spray appears if
not used for two consecutive days . Rinse the applicator and reprime with two
sprays or until a fine spray appears if not used for more than 14 days.
Budesonide is an anti-inflammatory corticosteroid that exhibits potent

glucocorticoid activity and weak mineralocorticoid activity. Corticosteroids
have a wide range of inhibitory activities against multiple cell types and
mediators involved in allergic and non-allergic-mediated inflammation. These
anti-inflammatory actions of corticosteroids may contribute to their effects in
seasonal and perennial allergic rhinitis.
The drug is indicated for the treatment of nasal symptoms of seasonal or

perennial allergic rhinitis in adults and children six years of age and older and
must be given nasally. The recommended dosage range in patients 6 to 11
years of age is 64 to 128 mcg per day
72
Contraindication:
This nasal spray is contraindicated for patients with hypersensitivity or allergic

to budesonide.
Side effects:
The common side effects are: nose/throat dryness or irritation, sore throat,
cough, sneezing, nosebleeds, unpleasant taste/smell, swelling, burning and
itching
Adverse effects:
The drug’s adverse effects are: loss of taste or smell, or pain or sores or white
patches in your nose.
Drug Interaction:
Caution should be observed when considering the co-administration of

Rhinocort Aqua Nasal Spray with long-term ketoconazole and other known
strong CYP3A4 inhibitors such as ritonavir, atazanavir clarithromycin.
RT Management:
Assess the patient’s medical history including allergies to the drug’s

ingredients especially with budesonide.
Consult the physician before use if the patient is using a steroid medicine for
conditions such as asthma, allergies or skin rash.
Rationale: To obtain baseline data from the patient
73
Assist the patient when administering the drug when necessary.
Rationale: To avoid overdosing or underdosing of the patient
Advise the patient to take the nasal spray at the same time each day
Rationale:Take the medication on regular intervals since its effectiveness
depends on its regular use.
Monitor effects and continue use if drug is effective for the patient
Rationale: Do not stop taking this medication without consulting the physician
and monitoring the effects of the drug.
Rationale:To give immediate treatment to the symptoms of the side and
adverse effects and allergy symptoms.
DRUG STUDY 10
Date Given: July 29, 2022
Brand Name: Maxipime
Generic Name: Cefepime
Classification: Fourth generation cephalosporins
Mode of Action:
74
Cefepime and other beta-lactam antibiotics have a similar mode of action. It
disrupts bacterial cell wall by binding and inhibiting transpeptidases known as
penicillin-binding proteins (PBPs), which are enzymes involved in the final
stages of peptidoglycan layer synthesis. This results in the lysis and death of
susceptible microorganisms. It also has a broad spectrum of in-vitro activity
which includes Gram-negative and Gram-positive bacteria. Cefepime has an
affinity for PBP-3 and PBP-1 in Pseudomonas aeruginosa, and PBP-2 in
Enterobacter cloacae and E. coli.
Contraindication:
Contraindicated in patients who have a previous hypersensitivity reaction to

any cephalosporin class of antibiotics, other beta-lactam antibiotics, and
penicillins.
Side Effects:
Abdominal or stomach cramps, back, leg, or stomach pain, bleeding gums,

nosebleeds, confusion, convulsions, dark urine, difficulty with breathing, fever,
chills, general body swelling, headache, irregular heartbeats, loss of appetite,
mood or mental changes, muscle cramps in the hands, arms, feet, legs, or
face, nausea or vomiting, numbness and tingling around the mouth, fingertips,
or feet, tremor, and yellowing of the eyes or skin.
Adverse Effects:
Pseudomembranous colitis, hypersensitivity (reaction-rash), urticaria, pruritus,

a transient elevation in ALT/AST, bilirubin, partial thromboplastin, anemia,
positive coomb’s test without hemolysis.
Drug Interaction:
75
Renal function should be monitored if high doses of aminoglycosides are to
be given with cefepime because of the increased potential of nephrotoxicity of
aminoglycoside antibiotics.
Cefepime administration may result in a false-positive reaction for glucose in

the urine when using Clinitest tablets.
Cefepime may reduce the efficacy of live bacterial vaccines such as typhoid
vaccines.
RT Management:
Determine if the patient has an immediate allergic response to beta-lactams.

Rationale: This is to make sure the patient will not have any allergic reactions.
Perform physical examination before starting drug therapy, this is to establish

baseline data, to assess the incidence of adverse effects related to beta-
lactams.
Rationale: To obtain baseline data from the patient and to not improvement.
Instruct patient to inform the physician about his/her medical history,

particularly intestinal/stomach diseases such as colitis and kidney disease.
76
Rationale: It is important to take into account the entire history of the patient
before proceeding.
Advise the patient to inform their physician that they are taking cefepime
before receiving immunizations or vaccinations. Cefepime may reduce the
effectiveness of live bacterial vaccines.
Rationale: This is to allow the physician to have a full medical record and
allow ample grace period before administering or even scheduling
vaccinations.
Instruct patients not to begin, adjust the dosage, or discontinue medication

without consulting their physician.
Rationale: Effectiveness of the medication can only be guaranteed if taken
properly.
Inform patient to take the medication exactly as prescribed to quickly see

improvements.
Rationale: This encourages the patient to stay on proper dosage.
77
DRUG STUDY 11
Date Given:
Brand Name: Ventolin
Generic Name: Salbutamol
Classification: Bronchodilator
Mode of action:
Works by engaging itself with the smooth muscles of all airways in order to
avoid constriction of the bronchioles.
Used to manage the symptoms of bronchospasm caused usually by bronchial

asthma but is easily also caused by other chronic bronchopulmonary
disorders.
Oral Inhalation: 2.5mg STAT prior to Hypertonic solution.
Contraindication:
Should be avoided for people with high blood pressure, pregnant or currently
breastfeeding, uterine disease, and hypersensitivity.
Side Effects:
78
Tremors of the hand, nervous tensions, headaches, palpitations, muscle
cramps.
Adverse Effects:
Nervousness, shakiness, throat and nasal irritation
Drug Interaction:
Risk of hypertension can be increased if Salbutamol is taken alongside

benzylimidazole, Dimethoxy-ethyamphetamine, Methoxyamphetamine.
Severity or risk of adverse effects for Abediterol may increase gradually if
taken alongside Salbutamol.
RT Management:
Assess the patient’s medical history to establish data for assessing

effectiveness of the drug and other adverse effects associated with the
specific drug.
Assess for possible allergies, cautions and contraindications

Rationale:To obtain baseline data for the patient and to determine
management for emergency situations like sudden onset of an allergy.
effects.
Rationale: By observing changes before and after the medication, immediate
care can be provided if adverse effects occur.
79
DRUG STUDY 12
Date Given:
Brand Name: Fortaz
Generic Name: Ceftazidime
Mode of action:
This drug is a bactericidal agent that works by inhibiting the bacterial cell wall
synthesis. The drug Ceftazidime has activity in the presence of some beta-
lactamases, both penicillinases and cephalosporinases, of Gram-negative and
Gram-positive bacteria.
Indicated for patients with : Lower Respiratory Tract Infections, Skin and Skin-
Structure Infections, Urinary Tract Infections, Bacterial Septicemia, Bone and
Joint Infections Gynecologic Infections, Intra-abdominal Infections and Central
Nervous System Infections
Contraindication:
80
This medication is contraindicated to patients who are hypersensitive to
ceftazidime or the cephalosporin group of antibacterial drugs.
Side Effects:
The common side effects are pain, swelling, burning, or irritation around the
IV needle, nausea, vomiting, diarrhea, stomach pain, and vaginal itching or
discharge
Adverse Effects:
Undesirable effects of this medication are hives, difficulty breathing, swelling

of your face, lips, tongue, or throat, severe stomach pain, diarrhea that is
watery or bloody, confusion, problems with speech or memory, seizures, and
cold feeling, discoloration, or skin changes in your fingers
Drug Interaction:
Nephrotoxicity has been observed after the administration of cephalosporins

with aminoglycoside antibacterial drugs or potent diuretics such as
furosemide.
RT Management:
Assess the patient’s medical history to establish data for assessing

effectiveness of the drug and other adverse effects associated with the
specific drug.
Assess for possible allergies, cautions and contraindications

Rationale:To obtain baseline data for the patient and to determine
management for emergency situations like sudden onset of an allergy.
81
effects.
Rationale: By observing changes before and after the medication, immediate
care can be provided if adverse effects occur.
Inform the patient that antibacterial drugs, including Fortaz should only be
used to treat bacterial infections as it doesn’t work with viral infections.
Rationale: Antibacterial drugs doesn’t work with viral infections
Assist the patient when administering the drug if necessary.

Rationale: To avoid overdosing or taking small doses of the medication.

Rationale: Monitoring the respiratory and breath sounds will help the RT to
reduce the side and adverse effects which may worsen.
Monitor pulse and blood pressure to evaluate cardiac response.

Rationale: Improvement in blood pressure and cardiac values should also be
noted
82
Inform the patient to seek professional help when experiencing undesirable
side effects or sudden onset of allergies as it may escalate into serious
problems.
Rationale: It may escalate into serious problems when left unattended.
DRUG STUDY 13
Date Given:
Brand Name:
Amikin
Amikin Pediatric
Generic Name: Amikacin
Classification: Aminoglycoside
Mode of action:
Amikacin, an aminoglycoside, binds to the prokaryotic ribosome, inhibiting

protein synthesis in susceptible bacteria. It is bactericidal in vitro against
Gram-positive and Gram-negative bacteria.
For patients in need of short-term treatment for strains of Gram-negative

bacteria infections
83
30 – 35 mg/kg/day to be administered intravenously or intramuscular.
Contraindication:
Hypersensitivity is the only contraindication, serious toxic reactions to

aminoglycosides are a clear contraindication because of the known cross-
sensitivities of patients to drugs belonging to this class.
Side effects:
Nausea Vomiting, Loss of appetite, Increased thirst, Rash, Injection site

reactions (pain, irritation, redness).
Adverse effects:
Skin rash, fever, headache, paresthesia, tremor, nausea, and vomiting.
Drug Interaction:
There are no drug interactions associated with Amikacin.
RT Management:
Determine if patient is hypersensitive to aminoglycosides

Rationale: This is to make sure the patient does have any allergic reaction to
the drug.
Make sure to inform the patient of the purpose of administering this drug
Rationale: An informed patient is less anxious and tensed.
84
Advise the patient to inform their physician that they are going to take
Amikacin
Rationale: This allows the physician to still have a full and complete medical
record.
Inform the patient of the importance of maintaining the proper dosage and
time of dosage.
Rationale: This is to inform the patient that skipping doses might alter the
effect of the drug.
Allow patient to get comfortable before administering

Rationale: This allows for less tension in drug administration.
RESPIRATORY THERAPY CARE PLAN 1
July 29, 2022
Name: T.L.C.
Age: 13 years old
Gender: Male
Height: 59 inches
Weight: 70.54 lbs (32 kg)
Physician: Dr. Guia Lauren Tarranza, RTRP, MD
Diagnosis: Acute exacerbations related to pulmonary infection secondary to

Cystic
Fibrosis
85
SUBJECTIVE
“Di jud ko ka ginhawag tarong.”, stated the patient upon admission. He was
also coughing with expectoration.
OBJECTIVE
DATE: July 29, 2022, 11:29 A.M.
A. Vital Signs
Time 7:00 9:00 11:00 1:00 PM 3:00 PM Normal Range

AM AM AM
Temp - - - 39℃ 38.1℃ 36.5℃-37.5℃
RR - - - 34 bpm 28 bpm 16-22 breaths per

minute
HR - - - 111 bpm 107 60-110 beats per

bpm minute
BP - - - 111/63 105/60 108/62 mmHg

mmHg
SpO2 - - - 92% 93% 95%-100%
86
Interpretation: The patient is febrile and hypertensive. SpO2 reading is not
within normal range throughout the entire shift, however, it gradually
increased towards the end of the shift.
B. Neuro Vital Signs at 11:36 A.M.
Result Normal Level of

Consciousness
Eye Opening 3 4 - Spontaneous Lethargic
Verbal 4 5 - Oriented,
Response Appropriate
Motor 5 6 - Obeys commands

Response
PGCS Score 12 -
Moderate
Interpretation: A score of 12 in GCS is classified as moderate head injury.
General Survey
Male patient TLC, age 13, of Filipino-German ancestry was admitted to San
Pedro Hospital last July 29, 2022 because of pulmonary exacerbation
associated with cystic fibrosis. The GCS score taken upon admission was 12,
which shows that the patient was at a lethargic level of consciousness. Upon
inspection, the patient presents with digital clubbing indicating hypoxemia and
evident usage of accessory muscles for breathing which indicates increased
work of breathing. The patient also presented to be shorter for the average
height for his age and thinner as supported by his low BMI. Patient was put on
oxygen by nasal cannula and was given a small intravenous (IV) fluid bolus.
87
C. Cephalocaudal Assessment taken at 11:40 A.M.
Skin
Upon inspection, the patient's skin has increased perspiration levels.
Upon palpation, there are traces of crystallized salts forming against the
sweat glands. These presences of salt indicate the increased levels of
chloride which is significant for patients diagnosed with cystic fibrosis. The
higher level of salt in the perspiration of cystic fibrosis patients represent an
impairment in the electrolyte transport of the sweat glands (Yu & Sharma,
2021).
Head

Eyes
anicteric however it appears to be sunken, owing to his gradual loss of weight.
However, there are no signs of bleeding in the subconjunctival and retinal
Ears
Upon inspection, the external canthus of the eye aligns with a horizontal
line to join with the pinna of the ear. There are no notable lesions or
discharges, and the skin of the ear is uniform with that of the face.
88
Nose
Upon inspection, the nose of the patient is straight, symmetrical, in the middle,
and consistent in color, with some discharges that are typically associated
with a runny nose. There is also apparent nasal flaring which indicates
increased work of breathing. Nasal cannula at 2 L/min was administered.
Upon palpation, nasal polyps are present, which might indicate sinus infection
and congestion.
Mouth
Upon inspection, the lips of the patient appear to be slightly bluish and pale,
but with no apparent lesions. There is evident enamel hypoplasia and tooth
discoloration. In addition, an anterior open bite can be observed which can be
associated with nasal and sinus obstruction (Abu-Zahra, et al., 2019).
Moreover, a shrill, coarse whistling sound can be heard, which is a clinical
manifestation known as wheezing.
Upon palpation, there is no notable inflammation and swelling around the

mouth.
Neck

Upon palpation, there are no notable swelling, inflammation, or masses noted.

Normal pulse is noted.
Thorax and Lungs
89
Upon inspection, intercostal and subcostal retractions are evident. There is
evident use of accessory muscles which indicates the patient's increased
work of breathing. There are no apparent lesions and bruises present.
Upon palpation, the antero-posterior diameter of the chest is 1:1. Because of

the hyperinflation developed from cystic fibrosis, the patient presents with a
barrel chest. There are no notable masses and lumps over the chest. There is
no presence of a bounding pulse when palpated.
Upon percussion, dullness can be noted. This indicates the presence of

consolidation owing to the infection brought upon by cystic fibrosis.
Upon auscultation, normal heart sound can be heard at the fourth intercostal
space in the left midclavicular line, also regarded as the maximum impulse.
There is absence of normal breath sounds in the base, first and second
intercostal space, and in the trachea. Consequently, the patient presented
with high pitched wheezing during admission. There is also occasional
popping sound, known as crackles heard over the base of the lungs which
signifies accumulated secretions.
Abdomen
Upon palpation, the patient reports pain. Tenderness is noted.
Upon percussion, there is hyperresonance, which indicates that the

abdomen is filled with air. This indicates presence of gas for patients with
abnormal pancreatic insufficiency due to cystic fibrosis.
Upon auscultation, there are decreased bowel sounds. This can be
attributed to the patient's malabsorption caused by the pancreatic insufficiency
due to cystic fibrosis.
Upper extremities
Upon inspection, there are no IV lines connected with the patient except
for the nasal cannula delivering oxygen to the patient. There are no notable
90
Upon palpation, there is no warmness or coldness that can be felt on the
skin, bounding pulse is absent, and edema is not present.
Lower extremities
Upon inspection, there are no notable scars or bruises. Digital clubbing is
Laboratories
Date: July 29, 2022, 12:05 P.M.
Hemoglobin (g/dL) 9.4 11.4-15.4 ↓
RBC Count (x 10⁶ /µL) 3.20 3.80-5.20 ↓
MCV (fL) 93 78-89 N
MCH (pg) 29 27-31 N
MCHC (%) 33 32-36 N
RDW (%) 12.5 11.4-13.5 N
WBC count (x 10³ /µL) 14.8 4.5-12.5 ↑
Differential Count
91
Monocytes (%) 1.0 0-0.8 ↑
Eosinophils (x 10⁹ /µL) 0.28 0.10-0.20 ↑
Basophils (x 10⁹ /µL) 0.18 0.00-0.10 ↑
MPV (fL) 10.2 9-12 N
Implication: Hemoglobin and red blood cells are below the normal values
which conifrms presence of hypoxemia. Abnormally elevated values in WBC
count reveals an infection. Differential count results suggests infection due to
bacteria as neutrophils count shows elevated value beyond the normal range
and lymphocytes shows value below normal range which is a strong indication
of bacterial infection.
Date: July 29, 2022, 1:00 P.M.
ABG Element Result Result (Before end of shift) Normal Ranges
pH 7.49 7.47 7.38-7.42
PaCO2 52 mmHg 50 mm Hg 38-42 mmHg
HCO3 30 mEq/L 29 mEq/L 22-28 mEq/L
PaO2 62 mmHg 67 mm Hg 75-100 mmHg
92
Interpretation: Partially compensated metabolic alkalosis with uncorrected
hypoxemia
Implication: The patient’s ABG result shows partially compensated metabolic

alkalosis with uncorrected hypoxemia. It is partially compensated due to the
abnormally elevated value of PaO2 which shows that there is an attempt to
compensate the alkalosis. The blood pH is alkalotic as well as the HCO3
which indicates abnormality in the metabolic process.
E. Diagnostic Tests
Date: July 29, 2022, 12:10 P.M.
Sputum Culture
Result

e
Implication: Patient TLC is positive for Pseudomonas aeruginosa; an

opportunistic pathogen is known to cause infection in the urinary tract,
respiratory system, dermis, soft tissue, bacteremia, bone and joint,
gastrointestinal, and blood, especially in people with severe burns,
tuberculosis, cancer, cystic fibrosis, and AIDS. Furthermore, green sputum
correlates with pseudomonas infection like in, cystic fibrosis, bronchiectasis
and lung abscess.
93
IMAGING STUDIES
Chest X-Ray
Date: July 29, 2022, 12:20 P.M.
Source: emDOCs
Impression: Chest x-ray of the patient shows mucous plugging, peribronchial

thickening, and ground-glass opacities consistent with consolidation in the
alveoli of the lungs.
Implication: Chest radiograph of the patient reveals mucous plugging which

may indicate total or partial airway obstruction, bacterial infection or
inflammation. The thickening of the bronchial wall is also seen, which may
indicate inflammation of the airways and is a significant morphological sign
that appears in patients with cystic fibrosis. The presence of consolidation in
the alveoli may indicate that fluid (blood, exudate, transudate), cells tissue
and other material are building up in the airspaces of the alveoli.
F. Medications
Drug Dosage Date Taken
94
Antibiotic
Antipseudomonal fluoroquinolone 20 mL/kg 2 dose q12h

(Ciprofloxacin)
For double antibiotic therapy: Aminoglycoside + β-lactam
Aminoglycoside
Tobramycin 10mg/kg/day 1 dose QD
β-lactam
Cefepime 150-200 div q6h-q8h (max 6-8

mg/kg/day g/day)
Mucolytics ( 7% Inhaled hypertonic 4 mL q12h BID

saline)
Bronchodilator (Salbutamol) 2.5 mg STAT prior to

Hypertonic saline
Steroids (Inhaled steroids)
Beclometasone 400–1500 μg PRN
Budesonide 800 μg PRN
G.BIPAP
BiPAP Set Up
Parameters Initial Setting
95
IPAP 12 cmH2O
EPAP 5 cmH2O
RR 12 breaths/min
FIO2 100%
Analysis
Obstructive airway pattern related to pulmonary infection secondary to cystic

fibrosis as evidenced by worsening dyspnea, chronic coughing with
productive secretions of occasional blood-tinged or green sputum, fatigue,
mucous plugging as shown in the chest radiograph.
Planning
That within the 4 hours span of providing care, the patient will show
improvements on his breathing pattern as evidenced by:
Improve ABG values as evidenced by:
pH from to 7.49 to value within the normal range (7.38-7.42)
PaCO2 from 52 mmHg to value within the normal range (38-42 mmHg)
HCO3 from 30 mEq/L to value within the normal range (22-28 mEq/L)
96
PaO2 from 62 mmHg to value within the normal range (75-100 mmHg)
Improve vital signs as evidenced by:
Temperature from to 39 °C to value within the normal range (36.5°C – 37.5°C)
Respiratory rate from 34 bpm to value within the normal range (16-22 breaths
per minute)
Cardiac rate from 111 bpm to value within the normal range (60-110 bpm -
Awake)
Blood pressure from 111/63 mm Hg to value within the normal range (110-124
mmHg -Systolic; 70-79 mmHg -Diastolic)
SpO2 from 92% to value within the normal range (95%-100%)
Express alleviation or improvement from dyspnea
97
Improved oxygenation status
Lesser usage of accessory muscles when breathing
Implementation/ Intervention
Vital signs inde
Purse lip, behavioral alerations
Dependent Rationale
Initiate BiPAP during The body relies on the central nervous system to
bedtime control breathing while sleeping. Breathing becomes
more difficult when resting in a reclining position.
Using a BiPAP machine at night can assist to keep
the airways open. This promotes the exchange of
oxygen and carbon dioxide, making breathing
easier.
Administer prescribed
double antibiotic
therapy:
Tobramycin injection is used to treat serious
Aminoglycoside + β-
bacterial infections such as meningitis (infection of
lactam
the membranes surrounding the brain and spinal
cord) and infections of the blood, abdomen (stomach
area), lungs, skin, bones, joints, and urinary system.
Tobramycin
It works by blocking bacterial protein synthesis
98
This drug disrupts bacterial cell wall by binding and
inhibiting transpeptidases known as penicillin-
binding proteins (PBPs), which are enzymes
involved in the final stages of peptidoglycan layer
synthesis. This results in the lysis and death of
susceptible microorganisms.
Cefepime
Administer prescribed This drug is used to treat asthma and chronic

salbutamol. obstructive pulmonary disease (COPD) symptoms
such as coughing, wheezing, and shortness of
breath. It works by relaxing the muscles of the
airways of the lungs, making breathing easier.
A prescription drug used to treat electrolyte and fluid

replenishment symptoms. It is a source of water and
Nebulized prescribed
electrolytes. Hypertonic saline can be used alone or
7% hypertonic saline.
in combination with other drugs.
Redness and swelling are reduced by this drug.

Moreover, it aids in the treatment of inflammatory
Administer prescribed
conditions such as asthma and eczema.
steroids.
Perform ABG test. To monitor the acid-base level of the blood as well
as to to evaluate the effectiveness of pulmonary gas
exchange.
99
Provide supplemental A treatment that supplies you with additional oxygen
oxygen. to breathe, as some medical conditions might result
in low blood oxygen levels. Low blood oxygen levels
can cause shortness of breath, fatigue, and
confusion.
Perform airway Improve secretion mobilization, reducing airway

clearance therapy BID obstruction, infection, and inflammation.
Independent Rationale
Monitor patient’s vital This enables close monitoring of patient status and
signs every 2 hours. lets healthcare providers know if the medication and
therapy is working.
Assess and monitor To determine improvement in the clearance of

the patient's lung secretions.
sounds.
Check for nasal flaring To know whether therapy is effective in delivering

and usage of needed oxygen to the patient or whether it is lacking
accessory muscles. and the patient needs additional drive to breathe.
Determinant of increased work of breathing.
Any abnormalities Immediate interventions can be administered, and

should be reported to complications can be avoided.
the attending
physician.
Evaluation
Goal unmet.
100
After a span of 4 hours of providing care, the patient’s vital signs had only
slightly improved as evidenced by:
Temperature of 38.1℃
Heart rate of 109 bpm
Blood pressure of 108/60 mmHg
RR of 28 bpm to 34 bpm
SpO2 outside of normal range at 93%, but significantly higher compared to

the lower SpO2 prior to this finding.
ABG results did not reach the normal range but, compared to the previous
results, minimal improvements has been shown.
101
ABG Element Result (1:00 P.M.) Result (Before end of shift) Normal Ranges
pH 7.49 7.47 7.38-7.42
PaCO2 52 mmHg 50 mm Hg 38-42 mmHg
HCO3 30 mEq/L 29 mEq/L 22-28 mEq/L
PaO2 62 mmHg 70 mm Hg 75-100 mmHg
Dyspnea had been slightly reduced.
Oxygenation status improved moderately from 62 mmHg to 70 mmHg.
Upon inspection, the use of accessory muscle had slightly reduce.
Recommendations
Infection Control
Rationale: In both inpatient and outpatient settings, health care personnel

must take steps to avoid spreading pathogens from one patient to another.
Many of the germs that colonize the airways of children with cystic fibrosis are
contagious. Therefore, it is vitally important to maintain appropriate hand
hygiene.
102
Continue BiPAP during bedtime.
Rationale: The body relies on the central nervous system to control breathing
while sleeping. Breathing becomes more difficult when resting in a reclining
position. Using a BiPAP machine at night can assist to keep the airways open.
This promotes the exchange of oxygen and carbon dioxide, making breathing
easier.
Continue inhaled medications (antibiotic, HS, bronchodilators, steroids PRN).
Rationale: These medications helps to avoid the formation of thick, sticky

mucus, manage infection, and preserve lung function over time.
Continue providing supplemental oxygen
Rationale: To prevent hypoxemia-induced cellular hypoxia (low PaO2)
Continue performing airway clearance therapy (CPT)
Rationale: Improve secretion mobilization, reducing airway obstruction,

infection, and inflammation.
The vital signs of the patient should continue to be monitored every two
hours.
103
Rationale: This track the patient's progress while in the hospital, allowing for
the early discovery of delayed recovery or adverse occurrences. vital signs
provide basic parameters of a patient’s health status.
Continue monitoring blood gas values
Rationale: To monitor the acid-base level of the blood as well as to to

evaluate the effectiveness of pulmonary gas exchange. Furthermore, to check
for any improvements after respiratory intervention.
August 01, 2022
Name: T.L.C.
Age: 13 years old
Gender: Male
Height: 59 inches

Cystic Fibrosis
104
SUBJECTIVE:
“Medyo okay na akong paminaw. Makaginhawa na ko ug tarong.”, stated the

patient. He was still coughing with slight expectoration.
OBJECTIVE:
Vital Signs
Time 3:30 5:30 7:30 PM 9:30 PM 11:30 Normal

PM PM PM Range
Temperature 37℃ 37.2℃ 37.5℃ 37.7℃ 37.3℃ 36.5℃-

(Temp) 37.5℃
Respiratory 26 bpm 23bpm 25bpm 22bpm 23bpm 16-22

Rate (RR) breaths
per
minute
Heart Rate 107 97bpm 103bpm 111bpm 99bpm 60-110

bpm beats per
minute
Blood 105/60 102/59 104/61 108/63 107/62 108/62

Pressure mmHg mmHg mmHg mmHg mmHg mmHg
SpO2 93% 90% 92% 89% 94% 95%-

100%
105
Interpretation: The patient is afebrile and normotensive. SpO2 continues to
fluctuate throughout the entire shift.
A. Neuro Vital Signs at 3:42 PM
Result Normal
Eye Opening 4 4 - Spontaneous
Verbal 4 5 - Oriented, Appropriate

Response
Motor Response 6 6 - Obeys commands
PGCS Score 14 - Mild
Interpretation: The GCS score taken shows that the patient was at a mild level
of consciousness.
C. Physical Assessment
General Survey
Male patient TLC is awake and oriented, patient is connected to the nasal
cannula for continuous oxygenation. The patient presents mild digital clubbing
indicating hypoxemia and evident usage of accessory muscles for breathing
which indicates increased work of breathing.
Skin
106
Upon inspection, the patient does not appear to have pale skin as compared
to the previous days.
Upon palpation, there are still traces of crystallized salts forming against the
sweat glands.These presence of salt indicate the increased levels of chloride
which is significant for patients diagnosed with cystic fibrosis.
Head

Eyes
anicteric however it appears to be slightly sunken, due to his gradual loss of
weight. There are no signs of bleeding in the subconjunctival and retinal
Ears
Upon inspection, the external canthus of the eye aligns with a horizontal
line to join with the pinna of the ear. There are no notable lesions or
discharges, and the skin of the ear is uniform with that of the face.
Nose
and consistent in color, with some discharges that are typically associated
107
with a runny nose. There is also apparent nasal flaring which indicates
increased work of breathing. Nasal cannula at 2 L/min was administered.
Upon palpation, nasal polyps are present, which indicate sinus infection and
congestion.
Mouth
but with no apparent lesions. There is evident enamel hypoplasia and tooth
discoloration. In addition, an anterior open bite can be observed which can be
associated with nasal and sinus obstruction (Abu-Zahra, et al., 2019).
Absence of shrill, coarse whistling sound or wheezing.
Upon palpation, there are no notable inflammation and swelling around the
mouth.
Neck


Thorax and Lungs
Upon inspection, intercostal and subcostal retractions are reduced including

the evident use of accessory muscles which indicates the patient's increased
work of breathing. There are no apparent lesions and bruises present.
Upon palpation, the antero-posterior diameter of the chest is 1:1. Due to

hyperinflation developed from cystic fibrosis, the patient presents with a barrel
chest. There are no notable masses and lumps over the chest. There is no
presence of a bounding pulse when palpated.
108
space in the left midclavicular line, also regarded as the maximum impulse.
Normal breath sounds can be heard inconsistently in the base, first and
second intercostal space, and in the trachea. There is also occasional
crackles heard over the base of the lungs which signifies accumulated
secretions.
Abdomen
Upon palpation, mild tenderness and moderate pain is noted.
Upon percussion, there is hyperresonance, which indicates that the

abdomen is filled with air. This indicates presence of gas for patients with
abnormal pancreatic insufficiency due to cystic fibrosis.
Upon auscultation, there are decreased bowel sounds. This can be
attributed to the patient's malabsorption caused by the pancreatic insufficiency
Upper extremities
Upon inspection, there are no IV lines connected with the patient except
for the nasal cannula delivering oxygen to the patient. There are no notable
Upon palpation, there is no warmness or coldness that can be felt on the
skin, bounding pulse is absent, and edema is not present.
Lower extremities
Upon inspection, there are no notable scars or bruises. Grade 2 digital
clubbing is present which indicates mild hypoxemia.
Laboratories
109

Hemoglobin (g/dL) 10.4 11.4-15.4 ↓
RBC Count (x 10⁶ /µL) 4 3.80-5.20 N
MCV (fL) 88 78-89 N
MCH (pg) 30 27-31 N
MCHC (%) 31 32-36 ↓
RDW (%) 12.4 11.4-13.5 N
WBC count (x 10³ /µL) 13.6 4.5-12.5 ↑
Differential Count
Monocytes (%) 0.9 0-0.8 ↑
Eosinophils (x 10⁹ /µL) 0.31 0.10-0.20 ↑
110
Basophils (x 10⁹ /µL) 0.12 0.00-0.10 ↑
MPV (fL) 11 9-12 N
Implication: Hemoglobin and hematocrit are above the normal values as a

compensatory reaction to the deteriorated lung function in patients with cystic
fibrosis causing hypoxia and partly also hypercapnia by increasing oxygen
affinity and concentration of hemoglobin to secure oxygen loading in spite of
the reduced oxygen pressure (PO2) in pulmonary capillaries. Abnormally
elevated values in WBC, neutrophils and a below normal value in
lymphocytes is a strong indication of bacterial infection.
pH 7.48 7.38-7.42
PaCO2 43mmHg 38-42 mmHg
HCO3 29mEq/L 22-28 mEq/L
PaO2 70mmHg 75-100 mmHg
Interpretation: Partially compensated metabolic alkalosis with uncorrected

hypoxemia
111

Implication: The patient’s ABG result shows partially compensated metabolic

alkalosis with uncorrected hypoxemia. It is partially compensated due to the
abnormally elevated value of PaO2 which shows that there is an attempt to
compensate the alkalosis. The blood pH is alkalotic as well as the HCO3
which indicates abnormality in the metabolic process.
E. Diagnostic Tests
Sputum Culture
Result

e
Implication: Patient TLC is positive for Pseudomonas aeruginosa; an

opportunistic pathogen is known to cause infection in the urinary tract,
respiratory system, dermis, soft tissue, bacteremia, bone and joint,
gastrointestinal, and blood, especially in people with severe burns,
tuberculosis, cancer, cystic fibrosis, and AIDS. Furthermore, green sputum
correlates with pseudomonas infection like in, cystic fibrosis, bronchiectasis
and lung abscess.
IMAGING STUDIES
112
Chest X-Ray
Date: July 29, 2022 (12:20 PM)
Source: emDOCs
Impression: Chest x-ray of the patient shows mucous plugging, peribronchial

thickening, and ground-glass opacities consistent with consolidation in the
alveoli of the lungs.
Implication: Chest radiograph of the patient reveals mucous plugging which

may indicate total or partial airway obstruction, bacterial infection or
inflammation. The thickening of the bronchial wall is also seen, which may
indicate inflammation of the airways and is a significant morphological sign
that appears in patients with cystic fibrosis. The presence of consolidation in
the alveoli may indicate that fluid (blood, exudate, transudate), cells tissue
and other material are building up in the airspaces of the alveoli.
F. Medications
113
Ciprofloxacin (Antipseudomonal fluoroquinolone): 20 mL/kg, oral, 2 dose q12h
Tobramycin (Aminoglycoside): 10 mg/kg/day, IV, 1 dose QD
Amikacin (Aminoglycoside): 30-35 mg/kg/day, IV, 1 dose QD
Ceftazidime (β-lactam): 200-400 mg/kg/day, IV, div q6h-q8h (max 8-12 g/day)
7% inhaled hypertonic saline: 4mL, inhaled, q12h BID
Salbutamol (Bronchodilator): 2.5 mg, inhaled, STAT prior to Hyperonic saline
Beclometasone (Steroids): 400–1500 μg, inhaled, PRN
Budenoside (Steroids): 800 μg, inhaled, PRN
114
G. Positive Pressure Ventilation
BiPAP @ 2 LPM
IPAP 12 cmH2O FIO2 100%
RR 17 breaths/min I: E ratio 1:2.5
T-low Inspiratory Time 1 second
EPAP 5 cmH2O Expiratory Time 2.5 seconds
Analysis
Obstructive airway pattern related to pulmonary infection secondary to cystic

fibrosis as evidenced by worsening dyspnea, chronic coughing with
productive secretions of occasional blood-tinged or green sputum, fatigue,
mucous plugging as shown in the chest radiograph.
Planning
That within the 24 hours span of providing care, the patient will show
improvements on his breathing pattern as evidenced by:
Improve ABG values as evidenced by:
· pH from to 7.49 to value within the normal range (7.38-7.42)
· PaCO2 from 52 mmHg to value within the normal range (38-42 mmHg)
· HCO3 from 30 mEq/L to value within the normal range (22-28 mEq/L)
· PaO2 from 62 mmHg to value within the normal range (75-100 mmHg)
Improve vital signs as evidenced by:
115
· Temperature from to 39 °C to value within the normal range (36.5°C –
37.5°C)
· Respiratory rate from 34 bpm to value within the normal range (16-22
breaths per minute)
· Cardiac rate from 109 bpm to value within the normal range (55-85 bpm
- Awake)
· Blood pressure from 111/63 mm Hg to value within the normal range

(110-124 mmHg -Systolic; 70-79 mmHg -Diastolic)
· Pulse pressure from 109 bpm to value within the normal range (50-90
beats per minute)
· SpO2 from 92% to value within the normal range (95%-100%))
Implementation/ Intervention
Evaluation: Goal partially met
Recommendations
August 4, 2022
116
Name: T.L.C.
Age: 13 years old
Gender: Male
Height: 59 inches

Cystic
Fibrosis
SUBJECTIVE
"Medyo nagluag na akong paghinga, murag mas nag-okay ang akong pamati
pero naa gihapon ko mga plema" as verbalized by the patient.
"Mas okay na ang iyahang paghinga labi na ug naka-inom na siya ug tambal.

Dili na pud kaayo siya putla ug luya dili kaparehas katung una siya gidala diri"
as verbalized by the patient's mother.
OBJECTIVE
A. Vital Signs
Time 7:00 AM 9:00 AM 11:00 AM 1:00 PM 3:00 PM Normal Range
117
Temp 37.3℃ 37℃ 37.1℃ 37.4℃ 37.5℃ 36.5℃-37.5℃
RR 24 bpm 24 bpm 22 bpm 25 bpm 22 bpm 16-22 breaths

per minute
HR 100 bpm 98 bpm 105 bpm 102 bpm 99 bpm 60-110 beats
per minute
BP 105/80 100/85 90/80 100/80 100/80 108/62 mmHg

mmHg mmHg mmHg mmHg mmHg
SpO2 88% 90% 91% 91% 92% 95%-100%
Interpretation: The patient is afebrile and normotensive. SpO2 reading is not

within normal range throughout the entire shift, however, it gradually
increases towards the end of the shift. This signifies presence of hypoxemia
B. Pediatric Glasgow Coma Scale
Result Normal
Eye Response 4 - Spontaneous 4 - Spontaneous
Verbal Response 5 - Oriented, Appropriate 5 - Oriented, Appropriate
Motor Response 6 - Obeys commands 6 - Obeys commands
118
PGCS Score: 15 - Normal
Interpretation: The patient's pediatric GCS confirms normal neurologic

response.
C. Physical Assessment
General Survey
The male patient is lying on the bed, awake and oriented, with a continuous
oxygen flowing in through the nasal cannula connected to him. Pulse oximetry
is hooked for continuous monitoring of the oxygen saturation.
Skin
Upon inspection, the patient's skin does not look pale compared to the
previous days.
Upon palpation, there are traces of crystallized salts forming against the
sweat glands.These presence of salt indicate the increased levels of chloride
which is significant for patients diagnosed with cystic fibrosis.
Head

distributed to the scalp and presents as oily.
Eyes
119
anicteric. The eyes of the patient do not appear to be as sunken anymore
compared to the previous days. There are no signs of discharges in the
subconjunctival and retinal areas. Eyelashes have a gentle outward curl and
are dispersed uniformly.
Ears
Upon inspection, the external ear and ear canal does not present any form of
tenderness and there is absence of swelling.

Nose
and consistent in color. Discharges are absent. Nasal flaring is absent. Nasal
cannula is connected at 2 L/min.
Upon palpation, nasal polyps are still present but tenderness and number
have decreased compared to the previous days.
Mouth
but with no apparent lesions. This is indicative of persistent hypoxemia.
Wheezing is absent.
Upon palpation, there is no notable inflammation and swelling around the

mouth.
Neck

vein distention. There is occasional use of accessory muscles which indicates
the patient's increased work of breathing, although intermittently.
120
Thorax and Lungs
Upon inspection, intercostal and subcostal retractions, as well as use of

accessory muscles are reduced compared to the previous days. There are no
apparent lesions and bruises present.
Upon palpation, the antero-posterior diameter of the chest is 1:1 indicative of

a barrel chest. There are no notable masses and lumps over the chest. There
is no presence of a bounding pulse when palpated.

space in the left midclavicular line. Normal breath sounds can be slightly
heard in the base, first and second intercostal space, and in the trachea.
There are occasional crackles heard over the base of the lungs which
signifies accumulated secretions.
Abdomen
Upon palpation, there is no notable pain and tenderness.
Upon percussion, there is hyperresonance, which indicates that the abdomen

is filled with air.
Upon auscultation, there are decreased bowel sounds. This can be attributed
to the patient's malabsorption caused by the pancreatic insufficiency due to
cystic fibrosis.
Upper extremities
Upon inspection, there are no IV lines connected to the patient. There are no
notable bruises or scars. Figure seems to be reappearing now, indicating the
patient gaining weight over the course of days.
121
Upon palpation, there is no warmness or coldness that can be felt on the skin,
bounding pulse is absent, and edema is not present.
Lower extremities
Upon inspection, there are no notable scars or bruises. Digital clubbing is still
D. Laboratory Tests
DATE: August 4, 2022 (7:09 AM)
Hemoglobin (g/dL) 11 11.4-15.4 ↓
Hematocrit (%) 40 34-45 ↑
RBC Count (x 10⁶ /µL) 4.90 3.80-5.20 N
MCV (fL) 93 78-89 N
MCH (pg) 29 27-31 N
MCHC (%) 34 32-36 N
RDW (%) 12.7 11.4-13.5 N
WBC count (x 10³ /µL) 12.8 4.5-12.5 ↑
Differential Count
Lymphocytes (%) 45 28-48 N
122
Monocytes (%) 0.8 0-0.8 N
Eosinophils (x 10⁹ /µL) 0.22 0.10-0.20 ↑
Basophils (x 10⁹ /µL) 0.09 0.00-0.10 ↑
Platelet count (x 10⁹ /µL) 320 139-320 N
MPV (fL) 10.3 9-12 N
Implication: The CBC test shows an increase in white blood cell count
because of the presence of bacterial infection. However, it is not as high as
the previous days because of the administration of antibiotics. The test also
shows decreased hemoglobin levels which confirms hypoxemia.
pH 7.46 7.38-7.42
Implication: Results reflect partially compensated metabolic alkalosis with

uncorrected hypoxemia. Partial compensation shows an attempt to
compensate for the alkalosis. Values are not as high as the previous sampling
and are nearing the normal range.
123
E. Diagnostic Tests
Findings: Patient TLC is still positive of traces for P. aeruginosa. This is a

common opportunistic pathogen that develops and lingers in
immunocompromised and lungs affected with cystic fibrosis.
Sputum Culture
Result
Positive Highly mobile, opportunistic
Chest X-Ray
124
Findings: Presence of sputum and consolidation in the lobes of the lungs.
Impression: Posteroanterior chest view reveals decreased sputum production

in the lobes of the lungs, however still present but in lesser amounts
compared to the previous CXR.
F. Medications
Ciprofloxacin (Antipseudomonal fluoroquinolone): 20 mL/kg, oral, 2 dose q12h
Tobramycin (Aminoglycoside): 10 mg/kg/day, IV, 1 dose QD
Amikacin (Aminoglycoside): 30-35 mg/kg/day, IV, 1 dose QD
Ceftazidime (β-lactam): 200-400 mg/kg/day, IV, div q6h-q8h (max 8-12 g/day)
125
7% inhaled hypertonic saline: 4mL, inhaled, q12h BID
Salbutamol (Bronchodilator): 2.5 mg, inhaled, STAT prior to Hyperonic saline
Beclometasone (Steroids): 400–1500 μg, inhaled, PRN
Budenoside (Steroids): 800 μg, inhaled, PRN
BiPAP Set Up
Parameters Initial Setting
IPAP 12 cmH2O
EPAP 5 cmH2O
RR 12 breaths/min
FIO2 100%
ANALYSIS
126
Ineffective airway clearance related to pulmonary infection secondary to cystic
fibrosis as evidenced by inability to remove airway secretions, hyperinflation
due to obstruction, lobar consolidation on the chest x-ray, and coarse crackles
heard upon inspiration.
PLANNING
That within 24 hours span of providing care, the patient will be able to improve
airway clearance as evidenced by:
Improved vital signs:
Temperature within the normal range (36.5°C – 37.5°C)
Respiratory rate within the normal range (16-22 breaths per minute)
Cardiac rate within the normal range (60-110 bpm - Awake)
Blood pressure value within the normal range (110-124 mmHg -Systolic; 70-
79 mmHg -Diastolic)
SpO2 from 92% to value within the normal range (95%-100%)
127
Express alleviation or improvement from dyspnea
Lesser usage of accessory muscles when breathing
Improved oxygenation status
INTERVENTIONS
Dependent Rationale
Initiate BiPAP The body relies on the central nervous system to control
during bedtime breathing while sleeping. Breathing becomes more
difficult when resting in a reclining position. Using a
BiPAP machine at night can assist to keep the airways
open. This promotes the exchange of oxygen and
carbon dioxide, making breathing easier.
Administer This allows the signs and symptoms of the disease to

medication as be solved. It is also important to monitor proper dosage,
prescribed by the administration, and timing most especially in the
physician. Double-Antibiotic Therapy as the primary core of the
plan.
Monitor arterial To monitor the acid-base level of the blood as well as to

blood gas values. to evaluate the effectiveness of pulmonary gas
128
exchange.
Provide To aid in the hypoxemia of the patient.

supplemental
oxygen.
Perform airway This helps in the removal of secretions located in the

clearance therapy apices of the lungs.
BID
Independent Rationale
Monitor vital signs This enables close monitoring of patient status and lets
every 2 hours. healthcare providers know if the medication and therapy
is working.
Assess and To determine improvement in the clearance of

monitor the secretions.
patient's lung
sounds.
Check for nasal To know whether therapy is effective in delivering

flaring and usage needed oxygen to the patient or whether it is lacking
of accessory and the patient needs additional drive to breathe.
muscles. Determinant of increased work of breathing.
EVALUATION
Goal partially met.
After a span of 24 hours of providing care, the patient was able to improve
airway clearance as evidenced by:
Improved vital signs:
129
Temperature of 37.5℃
Heart rate of 99 bpm
Blood pressure of 100/80 mmHg
RR of 22 bpm which is borderline at normal, however is relatively lower

compared to previous days.
SpO2 outside of normal range at 92%, but significantly higher compared to

lower SpO2 of previous assessment.
Less and intermittent usage of accessory muscles upon inspection;
Normal breath sounds can be heard upon auscultation;
130
Decreased sputum production in the lobes of the lungs; and
Improved complete blood count test results;
Hemoglobin Count = 11.0 g/dL
Erythrocytes = 4.90 g/dL
Leukocytes = 12.8 g/dL
Segmented Neutrophils = 72%
Lymphocytes = 45%
Monocytes = 0.8%
131
Eosinophils = 0.22 g/dL
RECOMMENDATIONS
Perform medical handwashing before and after being in contact with the
patient.
Rationale: For patients with cystic fibrosis who are prone to infection, it is
important that nosocomial exposure to other risk of infection should be limited.
In addition, this deters the spread of microorganisms.
Wear gloves and proper personal protective equipment.
Rationale: For patients stationed in the intensive care unit, nosocomial-

associated diseases will level the risk for mortality. This will also decrease and
stop further spread of microorganisms to patients who are susceptible for
infection.
Provide airway clearance therapy at least twice daily.
Rationale: Cystic fibrosis patients have increased secretions in their lungs

compared to patients without cystic fibrosis. To facilitate easier expectoration
of these secretions accompanied by proper intake of medication, airway
clearance therapy will enable clearance of sputum production.
Continue monitoring vital signs every 2 hours.
132
Rationale: This will allow the healthcare providers to know whether the
medications given and the therapy conducted is tolerable and responsive.
This aids in checking the patient's status and determining prognosis.
Monitor patient's CBC and sputum test.
Rationale: Checking the CBC and sputum test will let the healthcare providers
know if the recurrent pulmonary infection still exists and causes the pulmonary
exacerbations experienced by the patient. This helps in checking the patient's
status.
Check for signs of increased work of breathing.
Rationale: Cystic fibrosis patients will show signs of increased work of

breathing because of the consolidation of secretions in their lungs. Effective
treatment means that usage of accessory muscles will be decreased. This will
track the patient's progress with their treatment regimen.
PULMONARY REHABILITATION
A crucial part of cystic fibrosis treatment is pulmonary rehabilitation

care. This includes treatments, managing symptoms, planning routines,
pulmonary exercise and airway clearing procedures are included in
rehabilitation. Training, education, and improved behaviour are also included
in the program. Exercise tolerance, muscular strength, quality of life, and
nutritional condition are monitored well in order to help the patients benefit
with the results of the rehabilitation program. Techniques for clearing airways
have been a beneficial influence on mucous clearance.
This section will discuss the patient’s rehabilitation plan after he is

discharged. This consists of medications, exercises, treatment, health
133
teaching, outpatient, diet, and spirituality. These are given to the patient’s
parents or caregiver for supervision.
Medicine
Currently, there are no cure available for the treatment of cystic

fibrosis. Medications and treatment plans available today are focused on
addressing exacerbations brought by the disease. Various types of medicines
are taken to prevent further exacerbation of the disease.
 Childhood immunizations - Children should get their pneumococcal and

influenza vaccines at an early age. Patients are advised to take the
vaccines as they can.
 Antibiotics - The bacteria that cause lung infections in patients with cystic
fibrosis may be more successfully combated by more recent drugs.
Antibiotic therapy is now available as an outpatient procedure thanks to
oral and injectable antibiotics. Tobramycin (Tobi), an aerosolized antibiotic
that can be inhaled functions by enabling medication to enter a person's
airways directly.
 Other medications -These medications are used to help keep the airways
clear. Some medications that might be prescribed include:
 Theophylline
 Steroid inhalers
 Nonsteroidal anti-inflammatory drugs
 Mucus-thinning drugs that make mucus easier to cough up
 Bronchodilators such as albuterol (Proventil, Ventolin)
 Enzymes - People with cystic fibrosis may experience digestive issues

and may take digestive enzymes orally with meals to aid with food
digestion and nutrient absorption. A high-calorie diet and vitamin
supplements may also be recommended by the doctor.
Exercise
134
This is a must for patients with cystic fibrosis. It usually involves the
upper and lower extremity training, inspiratory muscle training and chest
physical therapy techniques. The ideal schedule for this is usually 4 to 12
weeks with supervision. The duration should be within the range of 30 to 60
minutes. However, patients must not exert too much effort to prevent
exacerbation of cystic fibrosis. Children with CF should not scuba dive,
skydive, bungee jump or do high-intensity activities at high altitudes (mountain
climbing). Children who have an enlarged liver or spleen should avoid
collision sports such as football, basketball and soccer.
 Aerobic exercises are preferred over resistance type exercises.
 Tele-exercises wherein participants are able to exercise with a group

through the use of devices like computer and television.
 Advise the patient to participate in social activities that involve a lot of

moving and action.
Treatment
As of this present time there is no known cure for Cystic Fibrosis but
there are numerous treatments that can lessen symptoms and improve quality
of life. Medicine to make mucus in the lungs thinner and easier to cough up.
 Bronchial airway drainage - People with cystic fibrosis require a method to

physically clear their lungs of heavy mucus. The person may ask for some
assistance, such as a parent or caregiver, gently pound or clap on his
chest or back to dislodge the mucus after lying down in a position that
helps drain mucus from the lungs. Others employ a mechanical precursor,
also referred to as an electric chest clapper. To help them cough up
secretions, some people may wear an inflatable vest with a machine
connected that vibrates rapidly. Bronchial airway drainage is required for
cystic fibrosis patients in both adults and children, at least one to four
times a day. (Healthline, 2021)
 Medicine to widen the airways and reduce inflammation.
 Antibiotics to present and treat chest infection.
135
 Medicines to help the person absorb food better.
 Special techniques and devices to help clear mucus from the lungs.
 Taking supplements and a strict diet to prevent malnutrition.
Health teaching
The context of cystic fibrosis should be thoroughly explained to patients

and their guardians. There should be enough knowledge about the disease
and how it progresses. People with cystic fibrosis should be encouraged to
participate in physical exercise like sports and other activities. In addition,
patients should observe the following as they go about their daily lives:
 Remind patients to wash his or her hands properly.
 Maintain a daily schedule of treatments.
 Encourage patients to avoid smoke, dust and dirt.
 Encourage patients to exercise regularly and drink more water.
 Outpatient
The decision to discharge a child from the paediatric unit should be

made after a careful assessment of their health, compliance with certain
requirements such as stable feeding and respiratory function, and education
of the patient's caregivers regarding disease management. A long-term
program that may enhance the overall health and lung function may be
suggested by the physician. Typically performed as an outpatient procedure,
pulmonary rehabilitation may involve:
 Physical exercise that may improve your condition
 Breathing techniques that may help loosen mucus and improve breathing
 Nutritional counselling
 Counselling and support
 Education about your condition
Diet
136
Generally speaking, a diet with 40% of the calories coming from fat is
advised. A person with CF frequently requires up to twice the daily caloric
intake suggested for healthy individuals. For the patient’s diet these are the
suggested diet plan that the can applied for
 Healthy eating and nutrition practices increase quality of life, maintain

good lung function, and assist people with cystic fibrosis (CF) manage
many of the GI issues the disease frequently causes.
 Along with fruits and vegetables, people with CF are typically urged to
consume as much high-calorie, high-fat, and high-salt cuisine as they
choose.
 People who have CF must also be careful with their fluid intake.
 Electrolytes should be added to water when it's hot outside or when you're
sweating from exercise. More frequent, small, high-calorie meals may be
required to assist a child with CF acquire enough calories since their
stomachs may take longer to empty, which can lead to feelings of fullness
(Pool, 2019).
Spiritual
Cystic fibrosis (CF) is a chronic, life-decreasing condition that

necessitates extensive coping. Some patients changed how they thought
about their illness, framing it as a part of a divine plan that required them to
follow.
 Treatment practices are related to spiritual belief.
 We propose linear regression models assessing the effects of spiritual

notions on exercise, nebulized medicine, and airway clearance.
 Spiritual convictions were connected to adherence factors and intentions.

For the purpose of providing person-centred care, there has to be a
greater awareness of the connection between spirituality and health
behaviours.
137
PROGNOSIS
Patient TLC, 13 years old, and of Filipino-German descent, was admitted to

the hospital because of an acute exacerbation owing to a recurrent pulmonary
infection secondary to cystic fibrosis. Upon admission, the patient displayed
dyspnea and increased work of breathing, and visible cyanosis. He was
immediately hooked to the nasal cannula at 2 L/min and was given a small IV
bolus.
The patient was already diagnosed with cystic fibrosis when he was 1 month
of age. Owing to a family heritage of CF patients and carriers, the patient is
already aware of the manifestation and progression of his disease process.
He used to incorporate a daily regimen including medications, enzymes, and
vitamins necessary in order to decrease pulmonary exacerbations of CF,
however, he was not able to maintain this lifestyle for the last two months
because of the family's financial constrictions. With the absence of the needed
medications and therapy for the patient, he had an acute exacerbation last
July 29, 2022 which rushed him to the hospital. Various diagnostic tests were
done which affirmed a diagnosis of recurrent pulmonary infection of P.
aeruginosa. This pathogen is a common infection for patients with cystic
fibrosis.
Although there were previous financial problems that the family faced, they
were able to provide the patient's hospital needs. They were able to access all
the needed diagnostic tests and medication which helped in the quick
management of the patient's exacerbating symptoms. Although the patient's
status improved, there is still notable impairment of the lungs mainly because
of the incurable nature of cystic fibrosis. However, symptoms are being
managed by the care, medication, therapy, and treatment given.
Based on the observations above, the RT student interns taking after this
case have decided to give the patient a good prognosis. This is mainly due to
the improving vital signs and CBC test results of the patient, as well as quick
management of the pulmonary symptoms of the patient. In addition, the
body's response to the care provided and the support of the patients are vital
elements in supporting the progression of handling the patient's illness.
138
Moreover, strong adhesion to lifestyle changes and treatment regimen was
noted and considered to be a vital sign in terms of improvement.
Respiratory Therapist Management
The following dependent and independent management options must be

observed to help the patient manage his lifestyle despite the prevalence of his
condition:
 Physical Assessment: The patient's vital signs should be monitored

regularly to assess for changes, most especially with his oxygenation
status. Signs of dyspnea, nasal flaring, cyanosis, and expiratory grunting
should also be noted to check whether the patient is experiencing
hypoxemia that cannot be managed by the medications and treatment
regimen he is currently undergoing.
 Assess clinical manifestations: Because of the nature of the disease

process of cystic fibrosis, the patient is more susceptible in developing
more complications and recurrent pulmonary infection. The status of the
patient should be frequently checked by his pediatrician and routine
diagnostic tests must be incorporated in his treatment plan.
 BiPAP: Usage of BiPAP improves the respiratory status of patients with

CF. It is ideal for long-term usage and provides respiratory comfort.
 Family Education: It is important that the family is aware about how the
disease progresses. They should be able to know whether there is an
incoming exacerbation attack. The family should also be committed to
giving the necessary medication and adjusting to the optimal lifestyle
changes for the patient. This is done so that the patient is able to perform
his daily activities despite the limitations imposed by his condition.
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A Case Study on

In Partial Fulfillment of the Requirements in

RT-124 Clinical Education 1

PEDIATRIC INTENSIVE RESPIRATORY CARE

GUIA LAUREN L. Tarranza, RTRP

Agustin, Joannes Lorenz

Boca, Princess Yliezah

Pumares, Denise Louise

Refuerzo, Audrey Lou

Sabatin, Christian Miguel

Servinio, Ionna Gabrielle

I. Cover Page ..................................................................................................1

III. Introduction ...............................................................................................4

IV. Objectives .............................................................................................….6

b.Specific Objectives ...................................................................................6

V. Definition of Diagnosis .............................................................................7

VI. Personal Data ......................................................................................…..9

b. Medical Data ......................................................................…....................9

VII. Patient History ..........................................................…..........................10

a. Past Health History....................................................…...........................10

b. Maternal Health History.............................................…...........................10

c. Present Health History..............................................…............................10

ii. Vital Signs..............................................…………...................…...............13

iii. Quiet examination ....................................…………................................13

iv. Hands-on examination .......................................…………......................15

IX. Anatomy and Physiology .......................................................................23

XI. Diagnostics .....................................................…....................................36

XII.Drug Study ..............................................................................................45

XIII. Respiratory Therapy Care Plan ........…...............................................57

XIV. Pulmonary Rehabilitation ...............................................................….90

XV. Prognosis .....................................................................................…......93

XVI. References ............................................................................................94

Pediatric intensive care is recommended whenever pediatric patients

Cystic Fibrosis is one of the most common genetic disorders. It is an

A. Past Health History

Patient TLC is a thirteen-year-old male of Filipino descent. He was

B. Maternal Health History

C. Present Health History

Time Results Normal Range

Temperature (Temp) 39℃ 36.5℃-37.5℃

Heart Rate 111 bpm 60-110 beats per minute

SpO2 92% 95%-100%

Pediatric Glasgow Coma Scale

Result Normal Level of

Eye Opening 3 4 - Spontaneous Lethargic

Motor Response 5 6 - Obeys commands

Male patient TLC, age 13, of Filipino-German ancestry was admitted to

Upon inspection, the patient's skin has increased perspiration levels.

Upon palpation, there are traces of crystallized salts forming against

Pupillary reflexes are normal and pupillary diameter is at 3 mm.

Upon palpation, there are no notable swelling, tenderness, or lumps. The

Upon inspection, the nose of the patient is straight, symmetrical, in the

Upon palpation, there are no notable inflammation and swelling around

Upon palpation, there are no notable swelling, inflammation, or masses

Thorax and Lungs

Upon inspection, intercostal and subcostal retractions are evident.

Upon palpation, the antero-posterior diameter of the chest is 1:1.

Upon percussion, dullness can be noted. This indicates the presence

Upon auscultation, normal heart sound can be heard at the fourth

Upon inspection, scars are absent.

Upon palpation, the patient reports pain. Tenderness is noted.

Upon percussion, there is hyperresonance, which indicates that the abdomen

Upon inspection, there are no notable scars or bruises. Digital clubbing is