CYSTIC FIBROSIS

A Case Presentation

Presented to

The Faculty of the College of Nursing

PHINMA University of Pangasinan

Dagupan City
 TABLE OF CONTENTS

Table of Contents

I.1Introduction

II. Patient Profile

III. AnaPhy

IV. Phathophysiology

V. Nursing Care Plan

VI. Drug Study

VII. Patient Education

2
I.qINTRODUCTION

Cystic fibrosis (CF) is an inherited disorder that causes severe damage to the lungs,

digestive system and other organs in the body. It affects the cells that produce mucus, sweat and

digestive juices. These secreted fluids are normally thin and slippery. But in people with CF, a

defective gene causes the secretions to become sticky and thick. Instead of acting as lubricants,

the secretions plug up tubes, ducts and passageways, especially in the lungs and pancreas.

Cystic Fibrosis (CF) is a rare condition among Asians and has not been reported in the

Philippines as of this time. The inclusion of this disease in the Philippines’ Expanded Newborn

Screening Program (ENBS) has provided this Filipino family the opportunity of early detection

and appropriate management of this condition that could ensure the survival of the proband and

his other surviving siblings. (Almonte et al, 2024)

While the prevalence of cystic fibrosis in the Philippines is unknown, its rarity in the

Asian population should not prevent general pediatricians from considering this condition in

children with respiratory, gastrointestinal, and pancreatic conditions. The Philippine Expanded

Newborn Screening program is an important tool to detect this condition so that further work up

and appropriate management can be done. Furthermore, a complete description of the various

genotypic mutations and its phenotypic presentations among the Filipino population should be

done to customize a CF panel and management that would be appropriate for the local

population. (Kim et al, 2024)

This case study presents the disease process, including its description, anatomical

presentation, physiological effects, nursing analysis, health promotion, and disease prevention of

cystic fibrosis disease. It also covers the related nursing assessment and potential nursing

interventions.

3
II – Patient Profile

Name: T.B.

Age: 6 years old

Sex: Male

Ethnicity: Caucasian

Chief complaint

“I noticed a let-down in T’s exercise tolerance level a week ago, and the last couple of

days his cough and sputum production have gotten much worse. When he started having

breathing problems, I brought him in immediately. T is normally a bubbly and lively little boy

and it is obvious when he isn’t feeling well. I think that he has another infection.” As verbalized

by the patient’s mother.

History of Present Illness

At 8 months old, the patient was diagnosed with Cystic Fibrosis. Recently, he has been

feeling more tired during play, has a cough that has gotten worse with dark-colored sputum, and

has lost 2½ pounds in weight over the last week due to a decreased appetite. His oxygen

saturation is currently at 87%, and he is receiving supplemental oxygen through a nasal cannula.

Past Medical History

The patient, a 6 lb-7 oz white male, was born 74 months ago to a 23-year-old mother

after an uncomplicated pregnancy and normal vaginal delivery. His Apgar scores were 8 and 9 at

1 and 5 minutes, respectively. At 30 hours post-delivery, he developed abdominal distension, bile

vomiting, and absent bowel movements. A second physical exam revealed a tense abdomen with

faint bowel sounds. Radiography confirmed distended bowel loops, and an exploratory

4
laparotomy revealed meconium ileus with distal ileum atresia, which was resected successfully.

He recovered without complications but was lost to follow-up after discharge. At 8 months, the

patient returned with failure to thrive, frequent pale and foul-smelling stools, and recurrent

bronchitis. Examination revealed a frail, malnourished child with crackles in the lungs.

Diagnostic workup showed a sweat chloride level of 99 meq/L, and a stool smear was positive

for fat, confirming cystic fibrosis (CF). Subsequent hospitalization showed pneumonia and

positive cultures for Pseudomonas aeruginosa and Staphylococcus aureus. After aggressive IV

antibiotic therapy, the patient recovered. Over the following six years, he was hospitalized

multiple times for respiratory infections, hemoptysis, and was diagnosed with bronchiectasis and

pancreatic insufficiency. His mother performs postural drainage three times daily, and he is

maintained on a high-calorie, high-protein diet supplemented with fat-soluble vitamins and iron.

Family History

 Father has hypertension

 Mother is a known carrier of CF

 The patient is the only child born to a 24 years old father and 23 years old mother

 A maternal uncle died at age 16 from pneumonia secondary to CF

Social History

 Patient lives with his father and mother and is in his first grade

 Father is a full-time evening custodian at a local community college

 Mother is currently a “stay-at-home mom,” but is also a registered nurse

 No pets

 Father smokes but only outside of the home

Review of Systems

5
  Patient complains of chest pain when coughing

 Reduced ability to perform usual daily activities due to SOB

 (-) vomiting, abdominal discomfort/pain, diarrhea, constipation, change in urinary

frequency, increase in thirst

Medications

 Aerosolized Tobramycin: 300 mg, twice daily (BID)

 Albuterol: 2.5 mg via nebulizer, three times daily (TID)

 Dornase Alfa: 2.5 mg via nebulizer, once daily (OD)

 Fluticasone Propionate: 100 ug, 1 puff, twice daily (BID)

 Prednisone: 4 mg orally, every 6 hours (Q 6h)

 Pancrelipase: 5200 Lipase units, 30,000 Protease units, and 21,008 Amylase units (or

similar units depending on the specific formulation), taken with each snack.

 Ferrous Sulfate:15 mg orally, every 8 hours (Q 8h)

 ADEK Multivitamin Pediatric Chewable Tablets:1 tablet orally, twice daily (BID)

 Patient has no known drug allergies (NKDA).

Physical Examinations and Laboratory Tests

General Assessment

The 6-year-old patient is thin and seems weak, with a tired and pale appearance. When

his oxygen cannula is taken off, he struggles to breathe and gasps for air. He is smaller than what

is typical for his age, and he is seated on the examiner’s table in the emergency room.

Vital Signs

 BP: 105/68 (sitting)

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  PR: 122 (regular)

 RR: 33 (labored)

 Temp: 98.4°F

 Weight: 29lbs

 Height: 3’4

 SaO2: 95% with 1.5 L Oxygen, 88% on room air

Skin

 Pale

 Cool to the touch, dry, and intact

 (-) rashes, bruises, and other unusual lesions

 Good turgor

Eyes, Ears, Nose, Throat

 Pupils equal at 3 mm, round, and reactive to light and accommodation

 Extraocularlar muscles intact

 Funduscopic exam unremarkable

 White sclera

 Conjunctiva pale and non-edematous

 TMs clear throughout, translucent, and without drainage

 Nares with dried mucus in both nostrils

 No oral lesions or erythema

 Secretions noted in posterior pharynx

Lungs

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  Crackles heard bilaterally in upper lobes

 Decreased breath sounds in lower lobes

 Wheezing noted without auscultation

 Right Lower Lobe (RLL) and Left Lower Lobe (LLL) dull to percussion posteriorly

Heart

 Tachycardic with regular rhythm

 (-) murmurs and rubs

 S1 and S2 normal

 (-) S3 and S4

Abdomen

 Abdomen soft (non-tender/non distended)

 (+) Bowel sound

 (-) Hepatosplenomegaly, masses, and abnormal sounds

Gastrointestinal Tract/Reproductive System

 Stool heme negative

 Normal penis and testes

Extremities

 Mild clubbing noted

 (-) cyanosis, edema, and femoral bruits

 Capillary refill with normal limits (WNL) at < 2 sec

 Radial and pedal pulses 2+ throughout

Neurologic System

8
  awake, alert, and oriented

 DTRs 2+

 No gross motor or sensory deficits present

 Somewhat uncooperative with full neurologic exam

Laboratory Blood Test Result

Sputum Culture Results

 (+) Pseudomonas aeruginosa, Stenotrophomonas maltophilia, and Staphylococcus aureus

Pulmonary Function

 FEV, 63% of predicted

Chest X-Rays

 Consolidation of lower lobes of both lungs consistent with double pneumonia

Peripheral Blood Smear

 Microcytic hypochromic red blood cells

Laboratory Findings

This following part offers an analysis of the outcomes from the laboratory tests and imaging

scans, emphasizing the unusual observations that indicate the patient’s present health condition.

General Assessment Results Interpretation

9
Vital Signs PR: 122 A heart rate of 122 bpm,

known as tachycardia,

signifies that the heart is

beating at a faster rate than

normal, often as a result of

respiratory issues.

RR: 33 An increased respiratory rate

of 33 breaths per minute

indicates that the patient is

breathing more rapidly than

usual, potentially due to

respiratory distress or lung

disease.

SaO2: 88% on room air An oxygen saturation level

of 88% while breathing

room air suggests that there

is an insufficient amount of

oxygen in the blood,

necessitating the use of

supplemental oxygen to raise

it to a safer level of 95%.

10
Skin Pale, cool to the touch, dry The pale and cool skin

and intact appearance may suggest

anemia or dehydration.

Additionally, the skin

dryness might be a symptom

of dehydration.

Lungs Crackles heard bilaterally in The occurrence of crackles

upper lobes sounds in the upper lobes

indicates the presence of

fluid or mucus in the lungs.

Decreased breath sounds in Reduced breath sounds in

lower lobes the lower lobes may suggest

that certain areas of the lung

are not fully expanding or

experiencing decreased

airflow, possibly due to fluid

or consolidation.

Wheezing noted without Wheezing signifies narrowed

auscultations airways, often observed in

asthma or bronchitis.

RLL and LLL dull to Dullness in the lower lobes

percussion posteriorly suggests possible

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 accumulation of fluid or

lung consolidation, which

could be due to pneumonia

or pleural effusion.

Neurologic System Somewhat uncooperative The patient’s cooperation

with full neurologic exam was somewhat limited,

potentially affecting the

precision of the neurologic

evaluation; however, in

general, no major neurologic

impairments were identified.

Laboratory Blood Test

Result

Sputum Culture (+) Pseudomonas The presence of

aeruginosa, Pseudomonas aeruginosa,

Stenotrophomonas Stenotrophomonas

maltophilia, and maltophilia, and

Staphylococcus aureus Staphylococcus aureus in the

sputum indicates bacterial

infections in the lungs.

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 Pulmonary function FEV, 63% of predicted Lung capacity, as indicated

by FEV at 63% of predicted,

is reduced, falling below

normal and reflecting

significant impairment in

lung function.

Chest X-ray Consolidation of lower lobes Areas of the lung filled with

of both lungs consistent with fluid or infection, indicated

double pneumonia by consolidation in the lower

lobes, are consistent with

double pneumonia.

Peripheral Blood Smear Microcytic hypochromic red The presence of microcytic

blood cells hypochromic red blood cells

suggests anemia, commonly

caused by iron deficiency,

where red blood cells are

smaller and less colorful

than normal.

III. AnaPhy

a. Anatomical Presentation

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 Figure1.1.

The lungs are essential organs for gas exchange, specifically for supplying oxygen to the

blood and removing carbon dioxide from it. The lung parenchyma, which has alveoli, alveolar

ducts, and bronchioles, and the lung airways, which are made up of bronchi and bronchioles, are

the two sections that make up the thoracic cavity. The right lung has three lobes, while the left

lung has two. The lungs also have a soft, spongy, and pinkish-gray appearance.

Anatomically the lungs are complex respiratory organs located in the thoracic cavity. In

order to accommodate the location of the heart, they are separated into two sections: the left

lung, which has two lobes (superior and inferior), and the right lung, which has three lobes

(superior, middle, and inferior). The trachea divides into two main bronchi, each of which

branches off into a smaller bronchiole and leads to a lung. The bronchioles lead in tiny air sacs

known as alveoli, which are used to absorb oxygen and release carbon dioxide. Gas exchange

takes place in the capillaries that surrounds each alveolus. The pleura, a double-layered

membrane that covers the lungs, helps in lowering friction when breathing. To maintain

everything in place and functional, they also have lymphatic channels, blood vessels, and elastic

tissues. In order to ensure that we get adequate ventilation and gas exchange during breathing,

the diaphragm and intercostal muscles work with the lungs to expand and contract.

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 Figure 1.2

Mutations in the CFTR gene cause thick, dried mucus in the airways of people with

cystic fibrosis (CF), which slows mucociliary clearance and allows bacteria like Staphylococcus

aureus and Pseudomonas aeruginosa to grow. This offers a favorable environment for chronic

respiratory infections, which cause inflammation, airway blockage, and bronchiectasis. As a

result, lung damage develops over time, and respiratory failure is the main cause of death for CF

patients. Because the thick mucus clogs the airways, there is a greater chance of infection and

airflow limitation, which increases the advancement of lung disease.

b. Physiologic Affectation

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 Mutations in the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) gene

cause Cystic Fibrosis, a hereditary genetic condition characterized by the formation of thick,

sticky mucus due to errors in the control of salt and water in and out of cells. The digestive

system and lungs are most affected, but other organs are also affected. Lung damage, chronic

inflammation, and respiratory infections are brought on by mucus obstructing airways in the

lungs. Malnutrition and slow growth are additional results of its blockage of the pancreatic

enzymes' release, that impacts the digestive system. The main consequences and complications

of cystic fibrosis includes:

Chronic Respiratory Infections. The thick mucus in the lungs traps bacteria like pseudomonas

aeruginosa, which leads to chronic infections and inflammation that eventually destroy lung

tissue.

Bronchiectasis. An infection causes the airways to permanently expand, which raises the risk of

lung damage and further infections as well as making it more difficult to discharge mucus.

Respiratory Failure. Chronic infections and inflammation cause the lungs to work poorly over

time, which frequently leads to respiratory failure, the main cause of mortality for people with

cystic fibrosis.

Pancreatic Insufficiency. Blockages in the pancreatic ducts limit the flow of digesting enzymes,

which is the reason behind diabetes associated with cystic fibrosis, delayed growth, and

malnourishment.

Liver Disease. Mucus buildup in the bile ducts can lead to liver inflammation and scarring

(cirrhosis), which may progress to liver failure in severe cases.

16
Infertility. Most men with CF are infertile due to blockages in the vas deferens, while women

may have reduced fertility because of thick cervical mucus.

Nasal Polyps and Sinus Infections. Mucus buildup in the upper respiratory system is a major

cause of chronic sinus infections and nasal polyps.

Osteoporosis. Patients with cystic fibrosis are more susceptible to osteoporosis and bone

fractures due to malabsorption of calcium and vitamin D.

Cystic Fibrosis-Related Diabetes (CFRD). Damage to the pancreas often leads to CFRD, a

common complication that affects glucose metabolism.

Lung Transplants. In advanced cases, lung transplants may be required to extend life in patients

with severe lung damage.

IV – Pathophysiology

Cystic Fibrosis (CF) arises from genetic mutations in the CFTR (Cystic Fibrosis

Transmembrane Conductance Regulator) gene. This gene is responsible for producing a protein

that facilitates the movement of chloride ions across cell membranes. When mutated, the CFTR

protein’s function is compromised, disrupting the balance of salt and water across membranes

and resulting in the formation of thick, sticky mucus in various organs.

In the lungs, this mucus impairs the normal function of cilia, which are responsible for

clearing the airways by moving mucus. This ciliary dysfunction leads to mucus blockage in the

airways, trapping bacteria and leading to persistent infections. These infections cause

inflammation and damage lung tissue, resulting in bronchiectasis—abnormal airway dilation—

17
further impairing respiratory function. Over time, the combination of infections, inflammation,

and mucus build-up results in progressive lung damage that can ultimately lead to respiratory

failure.

Thick mucus in the pancreas blocks the pancreatic ducts, stopping the release of digestive

enzymes into the intestines. This results in poor weight gain, fatty stools (steatorrhea), and

deficiencies in fat-soluble vitamins (A, D, E, and K) due to malabsorption of nutrients.

Prolonged blockage can also cause pancreatic damage, leading to diabetes or hyperglycemia in

some patients.

In the liver, thick mucus can block the bile ducts, causing a backup of bile. This backup

can lead to bile duct obstruction from gallstones and eventually result in liver damage, such as

cirrhosis.

In the intestines, mucus build-up can cause intestinal obstruction, such as meconium ileus

in newborns or distal intestinal obstruction syndrome (DIOS) in older patients. These

obstructions disrupt normal digestion and lead to malabsorption and nutrient deficiencies.

In the reproductive system, males often encounter vas deferens obstruction, leading to

infertility, while females may experience thick cervical mucus, which can hinder fertility.

The sweat glands are impacted as well. Sweat glands experience a malfunction in

chloride transport, leading to an overabundance of salt being lost through sweat. This can lead to

dehydration and disruptions in electrolyte levels.

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V, Nursing Care Plan

PHINMA University of Pangasinan College of

Health Sciences

NURSING CARE PLAN

19
 ASSESSMENT NURSING ANALYSIS PLANNING INTERVENTIONS RATIONALE EVALUATION

Short-Term Goals: Monitor Nutritional Status:

The patient is a 6-year-old male The patient is at risk for To manage the patient’s Evaluate the care by confirming
with cystic fibrosis, presenting imbalanced nutrition: less than  Regularly assess and condition effectively, it is that the patient’s nutritional
signs of malnutrition. body requirements due to  Improve Nutritional document the patient’s essential to monitor vital signs status has improved, as
Status: Increase the weight, height, and regularly. This allows for the
increased metabolic demands patient’s weight by at growth trends. evidenced by weight gain and
Subjective: and malabsorption related to early detection of any changes better appetite. Ensure that
least 1 lb and improve  Track daily caloric and
The parent of the patient reports cystic fibrosis. This risk is appetite within 1-2 protein intake to ensure that may indicate worsening of symptoms of malabsorption,
poor appetite and frequent, evidenced by the patient’s low weeks. adequacy. the patient’s status, enabling such as greasy stools, have
greasy stools, indicating possible weight and height, which fall timely interventions. decreased and that energy
fat malabsorption. below age-appropriate growth  Enhance Nutrient Administer Pancreatic Administering medications, levels have increased. Assess
Absorption: Achieve including antibiotics and
standards. The patient reports the effectiveness of pancreatic
a decrease in greasy Enzymes: pancreatic enzymes, addresses enzyme administration and
Objective: poor appetite and has frequent, stools and
The patient’s weight of 29 lbs greasy stools, indicating infections and supports dietary adjustments in
improvement in stool
and height of 3’4” are below malabsorption issues. consistency within 1  Ensure the patient digestion, while managing improving digestion and overall
expected growth standards. His Additionally, low energy levels week. receives prescribed blood pressure and relieving health. Monitor for signs of
skin is pale and slightly dry, but are noted, exacerbated by  Increase Energy pancreatic enzymes pain. Optimizing oxygenation progress, such as improved
turgor is good. The patient shows chronic respiratory problems. Levels: Improve the with every meal and is crucial to maintain adequate growth metrics and reduced
patient’s reported snack. oxygen levels and prevent
symptoms of malabsorption and Vital signs include a blood fatigue. Verify that the patient
energy levels and  Monitor for respiratory complications.
low energy levels, which are pressure of 105/68, a heart rate activity tolerance effectiveness and adjust and family have adhered to
exacerbated by his increased of 122 bpm, a respiratory rate Supporting fluid resuscitation dietary recommendations,
within 1-2 weeks. dosages as needed.
metabolic demands due to of 33 bpm, and an oxygen  Optimize Enzyme helps to improve blood volume enzyme use, and follow-up
chronic respiratory issues. saturation of 95%. These Use: Ensure Optimize Nutritional Intake: and tissue perfusion, which is appointments, and that
factors emphasize the urgent consistent vital for overall stability. education on managing cystic
Vital signs: need for targeted nutritional administration of Managing pain enhances the fibrosis and preventing
pancreatic enzymes  Provide high-calorie, patient’s comfort and
Blood Pressure: 105/68 interventions and respiratory complications has been
with every meal and high-protein meals and contributes to a more effective
Heart Rate: 122 bpm support to address the patient’s snack within 24 snacks that are tailored understood and implemented.
Respiratory Rate: 33 bpm physical and nutritional recovery. Addressing anxiety is
hours. to the patient’s
Oxygen Saturation: 95% requirements effectively. preferences and needs. important to reduce stress and
Long-Term Goals:  Offer frequent, smaller encourage cooperation with the
meals to improve treatment plan. Collaborating
NURSING DIAGNOSIS calorie intake and with the healthcare team
 Achieve Growth digestion. ensures that all aspects of the
Milestones: Reach patient’s care are coordinated
age-appropriate Manage Malabsorption: 20
The patient is at risk for and comprehensive. Finally,
weight and height
imbalanced nutrition: less than growth standards by educating the patient and
body requirements related to 3-6 months.  Monitor stool family improves their
 PHINMA University of Pangasinan College of
Health Sciences

NURSING CARE PLAN

NURSING ANALYSIS
ASSESSMENT PLANNING INTERVENTIONS RATIONALE EVALUATION

Subjective: Short-term Goals: Independent: Continuous monitoring will Short-term Goals:

"I noticed a let-down in T's exercise provide early detection of a
Impaired gas exchange is due After 8 hours of nursing worsening state of oxygenation After 8 hours of nursing
tolerance level a week ago, and the intervention, the patient: Monitor Vital Signs status and further guide intervention, the patient:
to too much sticky mucus that interventions in the treatment of
last couple of days his cough and 1. will report decreased the patient.
fills up the airways, and shortness of breath.
sputum production have gotten Proper positioning decreases
chronic infections and pressure on the diaphragm and 1. reported a reduction in
2. will demonstrate
much worse. When he started enhances lung expansion.
inflammation inside the lungs. improved breathing
Position the patient in the shortness of breath.
having breathing problems, I patterns, as evidenced by upright or semi-fowler's
Leads to decreased oxygen a reduction in labored position. Deep breathing exercises
brought him in immediately. T is breathing. promote lung compliance to 2. demonstrates decreased
delivery to the tissues, avoid atelectasis and improve the labored breathing and improved
normally a bubbly and lively little Encourage the patient to do necessary oxygen levels in a respiratory patterns.
increased carbon dioxide, and Long-term Goals: deep breathing exercises. patient's air spaces.
boy and it is obvious when he isn't A
respiratory acidosis. Most fter 1-2 days of nursing Goal was met.
feeling well. I think that he has intervention, the patient: Comfort measures would assist
clients are usually tachypneic in reducing the anxiety level of Long-term
another infection.” as verbalized by 1. will establish and Goals:
the patient, thereby reducing his
or show labored breathing; Ensure the patient has a respiratory rate and effort to
the patient’s mother. maintain baseline, normal comfortable environment. After 1-2 days of nursing
breathe, thus improving the
cyanosis is often reported overall breathing. intervention, the patient:
respiratory function, and
21
Objective: along with fatigue their lungs 1. maintained normal respiratory
Vital Signs: oxygen saturation. function and oxygen saturation
BP-105/68 (sitting) cannot adequately exchange The administering of antibiotics levels.
PR-122(regular) would target the eradication of
RR-33(labored) gases. It is achieved through the causative infection causing
Temp-98.4°F Dependent: lung consolidation and impaired 2. demonstrates effective self-
monitoring of the respiratory 2. will demonstrate gas exchange. management strategies to
Wt-29lbs
Administer antibiotics as manage lung infection and
Ht-3’4”
function, medication aimed at effective self- prescribed by the physician. Oxygen therapy will replace
SaO2-95%with 1.5 L Oxygen, impaired gas exchange and
88% on room air proper use of medications.
removing mucus, controlling management strategies increased oxygen requirements
Upon assessment client has:
Pale skin color infections, and educating for lung infection and Goal was met.
and therefore correct hypoxemia
Fatigue
Abnormal breath sounds patients on proper techniques impaired gas exchange by and facilitate the improvement of
NURSING DIAGNOSIS
to ensure maximum lung discharge. Administer supplemental gas exchange in general.
Impaired gas exchange related to
lung infection as evidenced by function. oxygen as ordered by the
labored breathing, abnormal breath
sounds and bilateral lung physician.
consolidation on the chest X-ray.
Physiotherapy to the chest or
nebulized medication can be
administered by respiratory
therapists that could further
facilitate the process of
elimination of secretions from
airways and improving lung
Collaborative: functions.

Collaborate with respiratory

therapy.

22
23
V1. Drug Study

PHINMA University of Pangasinan College of

Health Sciences

DRUG STUDY
NAME OF DRUG MECHANISM OF CONTRAINDICATIONS SIDE EFFECTS ADVERSE EFFECTS NURSING RESPONSIBILITIES
ACTION
GENERIC NAME Prednisone decreases Aggression The primary adverse effects Increase dosage when patient is subject to
inflammation via suppression Untreated serious infections. agitation of prednisone include stress.
Prednisone of the migration of blurred vision hyperglycemia, insomnia,
polymorphonuclear Documented decrease in the amount increased appetite, Taper doses when discontinuing high-
BRAND NAME leukocytes and reversing hypersensitivity. of urine hypertension, osteoporosis, dose or long-term therapy to avoid
increased capillary dizziness edema, adrenal suppression, adrenal insufficiency.
permeability. It also Varicella fast, slow, pounding, or cataracts, and delayed wound
Deltasone, Rayos, suppresses the immune irregular heartbeat or healing. Do not give live virus vaccines with
system by reducing the Administration of live or pulse immunosuppressive doses of
Other adverse reactions
Winpred activity and the volume of the attenuated live vaccine. headache corticosteroids.
include adrenal insufficiency,
immune system. The irritability
particularly when undergoing
antineoplastic effects may mood changes
stressful procedures or during Teaching points:
correlate with the inhibition noisy, rattling breathing
CLASSIFICATION sepsis; this is typically
of glucose transport, numbness or tingling in
diagnosable when the patient Do not stop taking the drug without
phosphorylation, or induction the arms or legs
is hypotensive and not consulting your health care provide.
of cell death in immature pounding in the ears
responsive to fluids,
lymphocytes. It may have swelling of the fingers,
vasopressors, or cardiogenic
antiemetic effects by blocking hands, feet, or lower
Glucocorticoids medications. Avoid exposure to infections.
the cerebral innervation of the legs
emetic center via inhibition of trouble thinking,

24
 INDICATION

Prednisone is indicated as
an anti-inflammatory or
immunosuppressive drug
for allergic, dermatologic,
gastrointestinal,
hematologic,
ophthalmologic, nervous
system, renal, respiratory,
rheumatologic, infectious,
endocrine, or neoplastic
conditions as well as in prostaglandin. speaking, or walking Report unusual weight gain, swelling of
organ transplant. There are trouble breathing the extremities, muscle weakness, black
a number of different After cell surface receptor
weight gain or tarry stools, fever, prolonged sore
attachment and entry into the
types, but those potentially throat, colds or other infections,
cell, prednisone enters the
used in people with cystic worsening of the disorder for which the
nucleus, binds, and activates
fibrosis are the group also drug is being taken.
specific nuclear receptors,
known as corticosteroids
or glucocorticoids. resulting in altered gene
expression and inhibition of
proinflammatory cytokine
DOSAGE & production. This agent
FREQUENCY decreases the number of
circulating lymphocytes,
inducing cell differentiation,
Prednisone 4 mg PO q 6
and stimulates apoptosis in
hours
sensitive tumor cell

25
 PHINMA University of Pangasinan College of
Health Sciences

DRUG STUDY

NAME OF DRUG MECHANISM OF ACTION CONTRAINDICATIONS SIDE EFFECTS ADVERSE EFFECTS NURSING RESPONSIBILITIES
GENERIC NAME Dornase alfa works by breaking Dornase alfa is Sore throat Pink eye Assess the patient’s respiratory
down DNA in the thick, sticky contraindicated in patients Fever Rash status, including lung function and
Dornase alfa mucus that accumulates in the who have had Stuffy nose Indigestion presence of mucus.
lungs of patients with cystic hypersensitivity reactions Chest pain Difficulty in breathing
BRAND NAME fibrosis. This enzyme is a from using any products Change in voice Check for any drug allergies and
recombinant form of human containing Chinese hamster educate the patient on dornase
Pulmozyme deoxyribonuclease I (DNase I), ovary cell products or alfa's purpose, potential side
which selectively cleaves ingredients. effects, and the importance of
CLASSIFICATION extracellular DNA released by consistent use.
degenerating neutrophils present
Recombinant enzymes in the mucus. By degrading this Administer dornase alfa via a
Mucolytic DNA, dornase alfa reduces the nebulizer while ensuring proper
viscosity of the mucus, making it patient positioning (upright for
INDICATION less thick and easier to clear from optimal lung expansion).
the airways. This helps improve
lung function and reduces the Monitor the patient during and
Dornase alfa Used as adjunct
risk of lung infections and after administration for tolerance
therapy in the treatment of
complications associated with and potential adverse reactions,
cystic fibrosis.
cystic fibrosis. including bronchospasm.
DOSAGE & FREQUENCY
Ensure the nebulizer is clean to
26
2.5 mg via nebulizer OD prevent infection and administer at
prescribed times, often alongside
chest physiotherapy.

Reassess the patient’s respiratory

status to evaluate drug
effectiveness.

Monitor for side effects such as

voice changes, rash, or serious
reactions like chest tightness.

Document the administration,

patient response, and any side
effects.

Reinforce proper nebulizer

hygiene and encourage adequate
hydration to help thin mucus.

27
VII. Patient Education

Patient Education

1.Encourage the parent to provide a diet high-calorie, and protein diet.

Rationale: Ensure that the patient receives adequate nutrition for appropriate weight and to

facilitate growth.

2. Instruct the parent that the child should take all medications as ordered.

Rationale: This will manage symptoms and complications will prevent. Some medications like

pancreatic enzymes help digest ingested food and mucolytics stimulate this process of clearing

the lungs from mucus.

3.Teach the family the use of CPT.

Rationale: The goal of CPT is to mobilize and loosen the mucus so it could be expectorated

from the lungs. This helps the child avoid complications like infections of the lung and the child

can breathe much easier.

4.Encourage the child to drink fluids and replace electrolytes.

Rationale: Keeps mucus thin, prevents dehydration in CF, fluid and salt balance is

compromised.

5.Teach the family to monitor for signs of respiratory infections or GI complications, including

increased cough or change in stool.

Rationale: Early detection of complications and early medical intervention prevent severe health

compromise.

6.Teach the family about the need for follow-up care with a CF specialist on a regular basis.

Rationale: Monitors disease process, and changes in treatment thus, maintains overall health.

7. Provide teaching on emotional support.

Rationale: Addresses the emotional concerns related to living with CF and improves coping

abilities by assisting them to communicate with others who have similar problems.

8. Encourage the child to attend school and participate in social activities within the limits of CF.

Rationale: The child is allowed to participate fully in life activities because the management of

CF does not make the child appear different from others.

9.Perform and instruct the child on deep breathing exercises.

Rationale: This promotes lung function, mobilizes mucus, and maintains patency of the airways.

10.Educate the family members regarding treatments and complication symptom awareness.

Rationale: The education will prepare all caregivers for the daily care of the child and allow for

quick response to any issues arising.

11.Educate the family on signs and symptoms that necessitate immediate medical attention,

including:

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Rationale: Symptoms such as these, if identified early and managed appropriately and in a

timely manner, will prevent serious health consequences and allow for adjustments in treatment

in a timely manner.

As a nursing student using the knowledge I have gained through my courses study, there

are a lot of great suggestions for a patient with Systic Fibrosis. It is very important that the

family and the patient themselves be made aware of all the resources that are available for

patients and families of those diagnosed with Cystic Fibrosis. Generally the information is shared

with the guardians of the patient, since young people are more affected and people than people

diagnosed later and life. The patient T.B is a 6 years old child who is not very well nourished. He

is underweight and his CF is present in his lungs.

Because of his age we will be teaching his patients and T.B, together about techniques

that could extend his life. Learning about things like chest breathing therapy and breathing

exercises to help clear sputum and mucus from the lungs. It’s very important to do daily airway

clearance exercises use inhaled medications as prescribed and using devices like nebulizer. Both

the patient and the guardians must understand that they have a responsibility in working with the

health care professionals to provide needed resources for the patient.

Knowing that CF affects nutrients absorption it will be important for me as a nursing

student to help both patient and guardians to learn the best dietary practices. Helping them learn

that a high-calorie, high protein diet in addition to enzymes supplements used before meals to

help with the digestion process along with vitamins and minerals. People with Cystic Fibrosis

have trouble observing fats, which means they have trouble absorbing vitamins that need fat to

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be absorbed such as A, D,E and K. This vitamins contain fat soluble and water soluble vitamins

to meet the needs of people with CF.

The CF patients will have lots of breathing and respiratory equipment to maintain and

keep clean. It is important that these accessories are kept clean and replaced as often as possible

as needed. The patient must also practice good hygiene, hand washing and avoiding close contact

with people who have respiratory infection. The wearing of N95 mask will also be a good

suggestion when they are outside of the home environment or they have a visitors in their home.

The patient should also be vaccinated against diseases that affect the lungs. As the

patient’s nurses it is important for us to encourage regular physical activity that is manageable by

the patient to help improve lung function, build strength and overall health. Any exercises must

be dependent upon the patient’s capabilities, emphasizing the way exercise helps in clearing

mucus. Breathing exercises will be the most important as the CF patient must fucos on clear

lungs.

As nurses working with CF patients we will be working with many other professionals in

the care of the CF patient, because CF is a life long condition. This disease will usually impact

that emotional health of the patient, because it generally is a death sentence. It doesn’t get better,

it will be important for the patient’s care team to discuss strategies to cope with their disease.

This strategies could include joining CF support groups and seeking mental health support to

help with stress, anxiety, or depression related to living with this disease.

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 REFERENCES

Bradbury, N. A. (2023). Cystic fibrosis. In Advances in Respiratory Medicine (pp. 563-588).

https://doi.org/10.1016/b978-0-12-821618-7.00159-0

Della Rocca, G., & Della Rocca, A. (2022). Cystic fibrosis. In Textbook of Respiratory Medicine
(pp. 729-740).
https://doi.org/10.1016/b978-0-323-71301-6.00050-0

Turcios, L. N. (2020). Cystic fibrosis lung disease: An overview. Respiratory Care, 65(2), 233-
251.
https://doi.org/10.4187/RESPCARE.06697

Raheel, S. A., & Bordoni, B. (2019). Anatomy, thorax, lungs. In StatPearls. StatPearls
Publishing.
https://www.ncbi.nlm.nih.gov/books/NBK541003/

West, J. B. (2012). Respiratory physiology: The essentials. Lippincott Williams & Wilkins.
https://pubmed.ncbi.nlm.nih.gov/22958998/

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