i

UNIVERSITY OF MINDANAO
COLLEGE OF HEALTH SCIENCES EDUCATION
Bachelor of Science in Nursing

In Partial Fulfillment

Of the Requirements in NCM109 Care of Mother and Child at-Risk

or with Problems
(Acute and Chronic)

A CASE PRESENTATION ON:

CYSTIC FIBROSIS

Submitted to:
Sarah Diana Rose S. Manalili, RN
___________________________________________________________________

Submitted by:

Deligero, Jean Mary Janer

Dema-ala, Renzly Tulio
Eñano, Jotham Lañojan
Flores, Claire Rabago
Geli, Anjewel Quibuyen
Jimenez, Gelmar Grace Brando
Lopez, Crizel Bartolome
Mamatas, Saima Baguio
Mandin, Caren Joy Reponte
Manlino, Janice Suan

Date of Submission:
June 28, 2021
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TABLE OF CONTENTS

CONTENT PAGES

I Cover page i
II Table of contents ii
III Description of the disease/illness 1-3
IV Definition of terms 3-4

V Etiology 4-7
VI Patient’s profile 7-13
VII Anatomy and Physiology of the affected body 13-23
parts
VIII Pathophysiology of the disease 24- 35
IX Medical management 36

Drug studies 36-58

Laboratory / Diagnostic Test 59-67
X Nursing management 68-78

XI Discharged plan 82-87

XII Health teaching 84

XIII Evidence-based research findings related to the 88-97

disease/illness
IX References 98-103
 1

Description of the disease

Cystic fibrosis (CF) is a chronic inherited disease that causes severe

respiratory and digestive system impairment. This injury is frequently caused by a

buildup of thick, sticky mucus in the organs. The lungs, pancreas, liver, and

intestines are the most often afflicted organs. The cells that generate perspiration,

mucus, and digestive enzymes are affected by cystic fibrosis. These produced fluids

are normally thin and silky, like olive oil. They lubricate different organs and tissues,

keeping them from drying out or becoming sick. This can result in life-threatening

complications like as infections, respiratory failure, and starvation. Cystic fibrosis is

unpredictable. In any event, hereditary testing should be done for couples with cystic

fibrosis or relatives who have the disease. Hereditary testing can determine a child’s

risk of cystic fibrosis by analyzing blood or spit samples from both parents. The

significance of the CF is Cystic fibrosis is a hereditary illness that causes recurrent

lung infections and gradually reduces one’s ability to breathe. Mutations in the cystic

fibrosis transmembrane conductance regulator gene cause the protein to become

dysfunctional in patients with the disease.

According to the Cystic Fibrosis Foundation Patient Registry More than

70,000 people globally are believed to have Cystic fibrosis, however the disease's

prevalence varies significantly around the globe. The prevalence of cystic fibrosis in

Canada and the United Kingdom is comparable to that found in the United States.

Approximately one in every 2,000 to 3,000 newborns in the European Union is born

with CF, which is somewhat higher than in the United States. Approximately 1,000

new cases of CF are diagnosed each year. More than 75 percent of people with CF

are diagnosed by age 2 and more than half of the CF population is age 18 or older.

The most affected group is Caucasians of northern European ancestry and the state

 2

of Ireland has the highest incidence of Cystic fibrosis. Some health problems caused

by CF can be treated, but the disease itself cannot be cured. Most people with CF

have a shortened life span; some will not survive past early childhood, but others will

live into their 40s or longer. The average survival of people with CF is about 36.5

years.

The Department of Health (DOH) in the Philippines deems an illness

uncommon when it strikes one client out of every 20,000 persons in the nation.

People with rare diseases may have a severely decreased quality of life and are

frequently dependent on others to meet their basic requirements. To ease the

disease's symptoms and effects, they also require lifetime medical care,

medications, and multidisciplinary therapy. It must be extremely unusual for cystic

fibrosis to exist in the Philippines. There is yet to be a sickness that has been clearly

diagnosed and confirmed.

Cystic fibrosis this is an inherited disease that found in the children. This

disease affects both male and female. A family history of cystic fibrosis is the most

important risk factor, especially if either parent is a known carrier. Cystic fibrosis is

caused by a recessive gene. This means that children must inherit two copies of the

gene, one from each parent, 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. The lungs and

pancreas are the most often damaged organs by cystic fibrosis, which can cause

respiratory and digestive difficulties. Mucus can still hold bacteria in a person with

cystic fibrosis, but it has difficulty migrating out of the lungs. As a result, germs can

accumulate in the lungs and cause severe illnesses.

 3

Symptoms of cystic fibrosis can differ from person to person, depending on

the severity of the condition. For example, one child with cystic fibrosis may have

respiratory but not intestinal issues, whereas another child may have both.

Furthermore, the signs and symptoms of cystic fibrosis may change with age.

Moreover, newborn screening (NBS) for cystic fibrosis is performed within the first

few days of life. By identifying Cystic Fibrosis early, Cystic Fibrosis health care

experts may assist parents in learning how to keep their kid as healthy as possible,

as well as postpone or avoid significant, lifelong health issues associated with Cystic

Fibrosis. Children who are identified Cystic Fibrosis early in life have better nutrition

and are healthier than those who are diagnosed later in life, according to research.

Early detection and treatment can improve development, keep lungs healthy, save

hospital visits, and add years to life. In addition, meconium ileus is a common initial

symptom of cystic fibrosis (CF) and affects around 20% of people diagnosed with

Cystic Fibrosis. It can manifest in two ways: simple meconium ileus and complex

meconium ileus. Viscid meconium physically obstructs the terminal ileum and small

intestine proximal to the blockage in simple meconium ileus, causing the intestine to

expand with more meconium, gas, and fluid. Complications such as prenatal

volvulus, ischemia necrosis, intestinal atresia, or perforation and ejection of the

meconium into the peritoneum can occur in complicated meconium ileus due to

meconium-distended portions of the ileum. The timing of perforation may have an

impact on the result. If the hole occurs earlier in the pregnancy, some meconium

may be reabsorbed in the peritoneum before birth, leaving just a few calcifications.

Definition of Term

Cystic fibrosis (CF) is an autosomal recessive, multisystem disease characterized by

altered transport of sodium and chloride ions in and out of epithelial cells. This defect
 4

primarily affects the lungs, GI tract (pancreas and biliary tract), and reproductive

tract. (Medical and Surgical Book)

Cystic Fibrosis (CF) a hereditary disorder affecting the exocrine glands. It causes the

production of abnormally thick mucus, leading to the blockage of the pancreatic

ducts, intestines, and bronchi and often resulting in respiratory infection. (Oxford

Languages)

Cystic fibrosis (CF) is a genetic disorder that affects mostly the lungs, but also the

pancreas, liver, kidneys, and intestine. Long-term issues include difficulty breathing

and coughing up mucus as a result of frequent lung infections. (Wikipedia)

Etiology

Cystic fibrosis is a hereditary disease that affects the production of mucus in

the body. Salt transport into and out of the cell is impaired by a genetic mutation in

the cystic fibrosis transmembrane conductance regulator (CFTR) protein, resulting in

thick, sticky mucus. Cystic fibrosis is caused by mutations (changes) in a gene on

chromosome 7, located on long arm at 31.2 band, one of the 23 pairs of

chromosomes that children inherit from their parents . The major apparent sign of the

illness is a blockage of the lungs' airways, as well as a lack of defensive action

against specific bacteria, which leads to infection. The sickness affects a variety of

body tissues and organs, resulting in a significantly decreased lifespan. Cystic

fibrosis was formerly considered a child's disease. The discovery of the disease's

fundamental causes has resulted in treatment breakthroughs that have substantially

improved survival rates. The illness is caused by mutations in the gene that codes
 5

for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This

protein is in charge of controlling the flow of salt and fluids into and out of cells all

over the body.

CFTR mutations may be divided into six functional categories, each of which

indicates a disruption in normal protein synthesis, trafficking, and function at the

epithelial cell membrane, or a combination of these issues. These mutations result in

a wide range of symptoms, from classic CF to single-organ involvement. To be

diagnosed with Cystic Fibrosis, you must have two copies of the CFTR gene, one

from each parent, with mutations. Both parents must have cystic fibrosis or be

carriers of a cystic fibrosis transmembrane conductance regulator (CFTR) gene

mutation for the child to be born. This indicates that if both parents should have

Cystic Fibrosis or have a CFTR gene mutation. Once two carriers have a child, the

youngster still has a 25% chance of developing Cystic Fibrosis. When one parent

has Cystic Fibrosis and the other is a

carrier, the child has a 50%

chance of developing the

disease.
 6

CFTR mutations may be divided into six functional categories, each of which

indicates a disruption in normal protein synthesis, trafficking, and function at the

epithelial cell membrane, or a combination of these issues. These mutations result in

a wide range of symptoms, from classic CF to single-organ involvement. Class I

mutations occur in the whole or partial loss of function of the CFTR protein, most

commonly as a result of a premature termination codon. Protein misfolding and a

lack of protein trafficking to the cell surface are caused by Class II mutations. When

the gating system fails to open in response to intracellular signals, Class III

mutations develop. The half-life of Class VI mutations is shorter. 13 In terms of

disease severity, individuals in group I–III had higher sweat test values than those in

classes IV–VI. Respiratory insufficiency or gastrointestinal (GI) issues are the most

common symptoms of Cystic fibrosis in newborns. The GI tract generally shows

signs of CFTR (Cystic fibrosis transmembrane conductance regulator gene) failure

before respiratory insufficiencies. Before the results of newborn screening are

known, meconium ileus will be symptomatic. Cystic fibrosis is commonly identified

before symptoms occur due to the present requirement for neonatal screening. A

chronic cough and wheeze are the most common symptoms in children, and they

are connected to GI malabsorption and growth failure. It's possible that infants have

meconium ileus, which can help with identification. Cystic fibrosis (CF) clients are

living longer and healthier lives than ever before, despite the fact that there is no

cure. In fact, today's CF babies are expected to survive well into their forties. Life

expectancy has increased to the point that there are now more people with cystic

fibrosis than children. Many medical advances in CF therapy, as well as client

 7

advocacy by groups like the Cystic Fibrosis Foundation, have contributed to this

progress (CFF).

Cystic fibrosis in the respiratory system is characterized by recurrent wheeze

or pneumonia, dyspnea during exercise, bronchiolitis, and hemoptysis. Common

gastrointestinal (GI) signs and symptoms include abdominal distension, steatorrhea,

biliary cirrhosis, and volvulus or intussusception. Discoveries in Cystic fibrosis

diagnosis and management have resulted in significant improvements in early

diagnosis and disease progression over time. The prognosis for clients is changing,

and supportive care becomes more commonly available. Primary healthcare

providers must be able to recognize CF illnesses and deliver apply suitable

treatment. Primary care providers must know how to evaluate and counsel clients in

order to slow disease development and help clients achieve the best possible quality

of life.

PATIENT’S PROFILE

Name (Initials/ Alias) : Client Z

Age : 12- years- old
Gender : Male
Weight : 27 kilograms
Civil Status : Children
Chief Complaint : Difficulty of Breathing
Admitting Diagnosis : Cystic Fibrosis
Admitting physician : Dr. Preni Pansaon
Attending physician : Dr. Mikee Siasu
Final Diagnosis : Cystic Fibrosis
 8

HEALTH HISTORY

The patient was diagnosed with cystic fibrosis. He suffers several years of

chronically productive cough with shortness of breath and wheeze. According to his

parents he developed a cough after his birth. At the age of five (5) years old, he was

first acutely unwell with a productive cough and weight loss. He was hospitalized and

improved the following of intravenous antibiotics. After three (3) years, he started on

asthma treatment following the development of chronic cough. His immunizations

were complete including BCG vaccination.

PRESENT HEALTH HISTORY

The patient is experiencing chronic productive cough with associated

shortness of breath and wheeze, also his chest had crackles upon auscultation and

had small lymph nodes. His vital signs are BP: 100/64 mmHg, Temperature: 37 ˚C,

Heart rate: 78 bpm, Respiratory rate: 25 bpm with a pulse oximetry of 98% on room

air. He also suspected asthma which he referred him on to secondary care that’s

why he started on taking Budesonide with Formoterol Preventer Inhaler (Symbicort)

and Terbutaline Sulfate Turbohaler (Bricanyl) to variable effect. He was treated for a

lower respiratory tract infection with 2 weeks of Co-amoxiclav (Amoxicillin with

Clavulanic Acid).

At follow-up, he was still symptomatic with chronically productive cough and

poor appetite. Further questioning revealed abdominal pain and steatorrhea.

Examination revealed finger clubbing, prompting further investigation into an

underlying chronic respiratory condition. Following above investigation, He started

on regular pancreatic enzyme replacement therapy, fat-soluble vitamins, nebulized

DNase and Prophylactic Flucloxacillin. He also receives dietetic and physiotherapy

 9

input. He had his first isolation of pseudomonas several months after diagnosis

which led to a 2-week admission for intravenous antipseudomonal antibiotics.

Following this, he was started on regular nebulized Colistimethate Sodium.

HEAD TO TOE ASSESSMENT

General Physical Assessment

Client Z, a 12-year-old boy, is awake, alert and conscious. There is no complaints of

fever, chills, and sweats. In addition, client’s appetite is “poor.”

Vital Signs:

Temperature: 98.6 F

BP: 100/64 mmHg

HR: 78 bpm

RR: 25 breaths per min with a pulse oximetry of 98% on room air.

Weight: 60lbs.

Skin, Hair, Nails

Upon examination client has salty skin. Sweat test revealed a high chloride level of

81 mmol/L, with >60 mmol/L reflecting a likely diagnosis of CF. Hair is normal.

Fingers are clubbing prompting further investigations into an underlying chronic

respiratory condition.

Head

Examination of the Head was normocephalic.

 10

Neck

No abnormalities were found.

Eyes

Eyes examination revealed client has strabismus. Eyes are not properly align with

each other when looking at an object.

Ears

No abnormalities were found.

Hearing

No difficulty in hearing.

Nose

Upon examination client has inflamed nasal passages or a stuffy nose.

Sinus

Upon examination, client has been experiencing recurrent sinusitis

Mouth

Client examination shows a poor dentition.

Thorax, Lungs, and Chest

Upon examination, yellow mucus, crackles, and gurgling in the chest were noted.

Client has poor inspiratory effort, and a productive cough.

Cardiovascular

There is no complaint of chest pain and dizziness. Upon auscultation, client has

regular rhythm without murmur or gallop.

Abdomen

Upon palpation, client has soft, non-tender, and no organomegaly. Client has

abdominal pain and poor appetite.

Upper Extremities
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Examination of upper extremities was normal.

Lower Extremities

Examination of lower extremities was normal.

Musculoskeletal System and Coordination

No difficulty and abnormalities noted. No balance issues.

Genitourinary

Client has no complaints of hematuria burning with urination. No foley catheter

attached.

Gastrointestinal

Client was neither nauseous nor vomiting. There is no change in bowel movement

but has steatorrhea.

Neurologic System

The client reaction level scale: GCS: 15. He was fully wake, alert and conscious.
 12

GENOGRAM

LEGEND:

Male

Female

Unaffected “carrier” of CF

With history of atopy

Affected male of cystic fibrosis

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INTERPRETATION

The diagram shows the medical history and relationship history of client Z’s

family. Starting from first generation (grandparents), second (parents), and third (the

client with CF). Client Z is diagnosed with Cystic fibrosis, an autosomal recessive

condition. This only means that his condition is genetics and inherited from his

parents. In order to have CF disease, each parent must carry one copy of the CFTR

mutated gene, but they typically do not show signs and symptoms of the condition.

Both paternal and maternal side looks normal because they are not affected

of the CF, but they are only “carrier” of a copy of mutated gene. The condition is not

expressed. In addition, his family history is positive for atopy. The two copies of CF

gene, each one from Z’s parents cause mutations in CFTR gene which resulted to

client Z’s disease.

ANATOMY AND PHYSIOLOGY

Cystic fibrosis is an autosomal recessive disease (genetic disease)

characterized by the buildup of thick, sticky mucus that can damage many of the

body's organs. It is caused by a mutation in the CF transmembrane conductance

regulator gene (CFTR) resulting in multisystem dysfunction. Mutations in the cystic

fibrosis transmembrane conductance regulator (CFTR) gene cause the CFTR

protein to become dysfunctional. When the protein is not working correctly, it’s

unable to help move chloride - a component of salt, to the cell surface. Without the

chloride to attract water to the cell surface, the mucus in various organs becomes

thick and sticky.

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CF affects 4 organ systems, including the following:

* Respiratory System

* Digestive System

* Integumentary System

* Reproductive System

Respiratory System

The respiratory system is the network of organs and tissues that helps to

breathe. It includes the airways, lungs, and blood vessels. These parts work together

to move oxygen throughout the body and clean out waste gases like carbon dioxide.

The respiratory system has many functions. Besides helping to inhale (breathe in)

and exhale (breathe out), it: allows you to talk and to smell, brings air to body
 15

temperature and moisturizes it to the humidity level your body needs, delivers

oxygen to the cells in your body, removes waste gases, including carbon dioxide,

from the body when you exhale, and protects your airways from harmful substances

and irritants.

Lungs

The lungs are a pair of spongy, air-filled organs located on either side of the

chest (thorax). The trachea (windpipe) conducts inhaled air into the lungs through its

tubular branches, called bronchi. The bronchi then divide into smaller and smaller

branches (bronchioles), finally becoming microscopic. The bronchioles eventually

end in clusters of microscopic air sacs called alveoli. In the alveoli, oxygen from the

air is absorbed into the blood. Carbon dioxide, a waste product of metabolism,

travels from the blood to the alveoli, where it can be exhaled. Between the alveoli is

a thin layer of cells called interstitium which contains blood vessels and cells that

help support the alveoli. The lungs are covered by a thin tissue layer called the

pleura. The same kind of thin tissue lines the inside of the chest cavity -- also called

pleura. A thin layer of fluid acts as a lubricant allowing the lungs to slip smoothly as

they expand and contract with each breath.

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In healthy lungs, there is a thin layer of mucus that helps your body move dirt

and bacteria out of the lungs. On the other hand, in people with Cystic Fibrosis (CF),

the abnormal electrolyte transport system causes the cells in the respiratory system,

especially the lungs, to absorb too much sodium and water. This causes the normal

thin secretions in the lungs to become very thick and hard to move. These thick

secretions, clogs the lungs, creating the perfect environment for harmful bacteria and

increase the risk for frequent respiratory infections.

Digestive system

Our digestive tract, which may also be called the “gastrointestinal tract” or “GI tract,”

is the route of food follows after you put it in our mouth. It is responsible for taking

whole foods and turning them into energy and nutrients to allow the body to function,

grow, and repair itself. The six primary processes of the digestive system include:

ingestion of food, secretion of fluids and digestive enzymes, mixing and movement of

food and wastes through the body, digestion of food into smaller pieces, absorption

of nutrients and excretion of wastes. In the digestive system, Cystic Fibrosis (CF)

mainly affects the pancreas, liver and the intestines.

 17

The Pancreas

The pancreas is a long, slender organ, most of which is located posterior to

the bottom half of the stomach. The pancreas has digestive and hormonal functions:

the enzymes secreted by the exocrine gland in the pancreas help break down

carbohydrates, fats, proteins, and acids in the duodenum. These enzymes travel

down the pancreatic duct into the bile duct in an inactive form. When they enter the

duodenum, they are activated. The exocrine tissue also secretes bicarbonate to

neutralize stomach acid in the duodenum. This is the first section of the small

intestine. The main hormones secreted by the endocrine gland in the pancreas are

insulin and glucagon, which regulate the level of glucose in the blood, and

somatostatin, which prevents the release of insulin and glucagon.

People with CF have sticky mucus that blocks ducts in the pancreas and

prevents enzymes from reaching the small intestine to digest food. Undigested food

in the intestines can cause pain, cramping, gas and either loose, greasy, floating

stools or constipation and blockages. Also, everyone with CF (including people who

don't need enzyme supplements) has a pancreas that does not make enough

bicarbonate to neutralize stomach acid. This can also contribute to pain, cramping,

gas and constipation. Bloating and excessive gas also can be caused by small bowel

overgrowth, gastric paresis and gastroesophageal reflux disease.

 18

The Liver

Liver is a roughly triangular organ that extends across the entire abdominal

cavity just inferior to the diaphragm. Among its many functions, the liver makes a

fluid called bile that helps the body absorb fat. Bile travels through small tubes or

ducts in the liver and is stored in the gallbladder, which empties the bile into the

small intestine - important function in digesting fat and some vitamins. As the mixture

of food, pancreatic fluid, bile and pancreatic enzymes moves along the small

intestine, the important nutrients make their way into the body by absorption through

special cells in the walls of the small intestine.

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In some people with CF, bile gets thick and flows very slowly. It can even get

so thick that it forms stones in the gallbladder, which sometimes need to be

removed. Cystic fibrosis can lead to liver disease by causing mucus to build up and

block bile ducts in the liver. This prevents bile from leaving the liver, which causes

inflammation and produces scarring (fibrosis). In severe cases, this scarring can

become permanent, a condition called cirrhosis. As a result, the liver cannot function

properly.

The Intestines

The intestines are a long, continuous tube running from the stomach to the

anus. Most absorption of nutrients and water happen in the intestines. The intestines

include the small intestine, large intestine, and rectum. The small intestine (small

bowel) is about 20 feet long and about an inch in diameter. Its job is to absorb most

of the nutrients from what we eat and drink. Velvety tissue lines the small intestine,

which is divided into the duodenum, jejunum, and ileum. On the other hand, the large

intestine (colon or large bowel) is about 5 feet long and about 3 inches in diameter.

The colon absorbs water from wastes, creating stool. As stool enters the rectum,

nerves there create the urge to defecate.

In a person with cystic fibrosis, the thick, sticky mucus blocks ducts (or paths)

between the pancreas and the intestines. It prevents enzymes that digest fats and

proteins from reaching the intestines. As a result, people with cystic fibrosis have

trouble digesting food and getting the vitamins and nutrients they need from it.
 20

Without these digestive enzymes, the intestine can’t absorb the necessary nutrients

from food. This can result in greasy, foul-smelling stools, constipation, nausea, a

swollen abdomen, loss of appetite, poor weight gain in children and delayed growth

in children.

Integumentary System

The integumentary system is the largest organ of the body that forms a

physical barrier between the external environment and the internal environment that

it serves to protect and maintain. The integumentary system includes the epidermis,

dermis, hypodermis, associated glands, hair, and nails. In addition to its barrier

function, this system performs many intricate functions such as body temperature

regulation, cell fluid maintenance, synthesis of Vitamin D, and detection of stimuli.

The Sweat Gland

Sweat glands, also known as sudoriferous or sudor parous glands, from Latin

sudor 'sweat', are small tubular structures of the skin that produce sweat. Sweat

glands are a type of exocrine gland, which are glands that produce and secrete

substances onto an epithelial surface by way of a duct. Sweat glands are located

deep within the skin and primarily regulate temperature. Sweat glands cool the body

by releasing perspiration (sweat) from the lower layers of the skin onto the surface.

Sodium and chloride (salt) help carry water to the skin's surface and are then

reabsorbed into the body. As the water evaporates, heat is carried away, and the

body cools.
 21

In people who have cystic fibrosis, the salt travels to the skin's surface with

the water and is not reabsorbed. Because of this, the skin of a child who has cystic

fibrosis is abnormally salty. Parents may notice salty-tasting skin when they kiss the

child. People who have cystic fibrosis can become quickly depleted of salts,

especially when the weather is hot, when they exercise strenuously, or when they

have a fever. Low salt levels in the body lead to fatigue, weakness, fever, muscle

cramps, stomach pain, vomiting, dehydration, and heatstroke.

Reproductive System

The reproductive system of an organism, also known as the genital system, is

the biological system made up of all the anatomical organs involved in sexual

reproduction.

Male Reproductive Tract

The Vas Deferens

The vas deferens is a long, muscular tube that travels from the epididymis into the

pelvic cavity, to just behind the bladder. The vas deferens transports mature sperm
 22

to the urethra, the tube that carries urine or sperm to outside of the body, in

preparationfor ejaculation.

Most men with CF (97-98 percent) are infertile because of an absence of the

sperm canal, known as congenital bilateral absence of the vas deferens (CBAVD).

The sperm never make it into the semen, making it impossible for them to reach and

fertilize an egg through intercourse. The absence of sperm in the semen can also

contribute to men with CF having thinner ejaculate and lower semen volume.

Female reproductive Tract

The Cervix

The cervix is a cylinder-shaped neck of tissue that connects the vagina and

uterus. Located at the lowermost portion of the uterus, the cervix is composed

primarily of fibromuscular tissue. The cervix performs two main functions: It facilitates

the passage of sperm into the uterine cavity and maintains sterility of the upper

female reproductive tract. The cervix, and all structures superior to it, are sterile. This

ultimately protects the uterine cavity and the upper genital tract by preventing

bacterial invasion. This environment is maintained by the frequent shedding of the

endometrium, thick cervical mucus and a narrow external.

 23

Women with cystic fibrosis have thicker cervical mucus due to abnormal cystic

fibrosis transmembrane conductance regulator (CFTR) function. Thicker mucus can

make it harder for sperm to successfully penetrate the cervix and can increase the

amount of time it takes to become pregnant.

CFTR ions channel

The CFTR protein is an ion channel, which is a type of protein. An ion channel

transports electrically charged atoms or molecules from inside the cell to the outside,

or from the outside to the inside. The CFTR ion channel in the lung transports

chloride ions from within to outside the cell.

The chloride ions exit the cell by passing through the middle of the tube

created by the CFTR protein. Once outside the cell, the chloride ions attract a layer

of water. This water layer is critical because it permits cilia, small hairs on the

surface of lung cells, to sweep back and forth. Mucus is moved up and out of the

airways with this sweeping motion.

In people with CF, mutations in the CFTR gene can cause problems with the

CFTR protein: it doesn't work well, it isn't produced in sufficient quantities, and it is

not produced at all. When any of these issues arise, chloride ions become stuck

inside the cell, and water is no longer drawn to the space outside the cell. The

mucus in the airways becomes dry and thickens when there is less water outside the

cells, flattening the cilia. When thick, sticky mucus clogs the cilia, they can't sweep

correctly.

Mucus is caught in the airways because the cilia can't move properly, making

breathing difficult. Furthermore, microorganisms captured in mucus are no longer

 24

evacuated from the airway, giving them the opportunity to grow and cause infections.

Some of the most common difficulties that patients with CF confront are thick mucus

in the lungs and frequent airway infections.

 Pathophysiology
Predisposing Factors Precipitating Factors
Cystic fibrosis 25
Inherited two copies of CFTR Knowledge deficit about the
mutated gene importance of taking a genetic
screening before conception

CFTR GENE DEFECT

DEFECTIVE ION TRANSPORT

ABNORMAL EXOCRINE FUNCTION

Respiratory Digestive Reproductive Integumentary

Lungs Pancreas Intestine Liver Male Female Sweat glands

Missing inhibitory CF has sticky Mucus builds up Mutation of CFTR Thick secretion of Due to the missing CFTR
effects of CFTR mucus that blocks Pancreatic enzyme and blocks bile gene causes the cervical mucus protein, chloride ions are
protein to the ENaC ducts in the are not released in ducts in the liver vas deferens to not reabsorbed
pancreas intestine atrophy early in
embryonic Mucus that coats
Prevents bile from Increased salt in sweat on
development the vagina and
Prevents the Undigested food the skin
leaving the liver cervix is thicker
Absorb too much enzymes in Congenital Bilateral than usual, making
sodium and water reaching the Steatorrhea Absence of Vas
intestine Excessive sweating
Deferens (CBAVD)
Secretions in the Small intestines Nutrients are not Damage to the liver it more difficult for Sweat test revealed high
lungs becomes very absorbed in the cells The sperm never sperm to travel and chloride level
to digest food 26
thick and hard to small intestine make it into the fertilize the egg
move Protease- semen, making it >60 mmol/L
responsible to Cirrhosis impossible for them
digest protein to reach and fertilize
Thick mucus clog an egg through
Amylase- Causes Ovulation issues
(obstruct) the airways Nursing intercourse Hyponatremia,
responsible to inflammation and due to poor
Intervention hypochloremia
digest produces scarring. nutrition
carbohydrates As a result, the liver
Drinking plenty of
cannot function Nursing Intervention
Dyspnea, wheezing, fluids (8-10
Lipase- properly. Takes a long time
and chest tightness glasses a day) Hydrocele (fluid Eat high in sodium foods
responsible to to get pregnant
accumulation like ham, butter,
digest fats Reducing dietary around the testes vegetable juices, and
fiber intake Increased portal
which can decrease processed cheese
Environment for hypertension
how well the testes Nursing
bacteria to grow Pancreas does Reducing dietary Intervention
not make enough work) Avoid excessive activities
fat intake
Jaundice, ascites, like biking, running,
bicarbonate to Further
Infection and Internal bleeding, jumping ropes and tteam
neutralize investigation of the
inflammation small vein ruptured sports like baskitball
stomach acid vaginal discharge
Medical
Nursing Checking vaginal
Damage to the lung treatment/manag Medical treatment
Impaired Intervention pH (You hold a
tissues ement /management
digestion piece of pH paper
Because bleeding against the wall of
Pancreatic Intravenous fluid,
can occur, monitor your vagina for a electrolyte replacement
Bronchiectasis, enzyme
Loss of appetite & the patient closely Nursing few seconds, then
pneumonia, replacement
abdominal pain for signs of Intervention compare the color
hemoptysis therapy (PERT)
hypovolemia. Test of the pH paper to
any stool and Checking for
Enzyme destroy the color on the If treated:
Nursing Intervention If treated: emesis for blood. tenderness in an
pancreatic cells chart provided with
enlarged scrotum
Ensuring the room is the test kit. Normal electrolyte levels
Improve digestion Instruct the patient
well ventilated for cutting out the Assess men during
Leads to High vaginal swab
Improve appetite salt from his/her assessment and
Monitor respiration inflammation and (HVS)
diet screening
and breathe sound scarring If not treated:
If not treated:
Take diuretic such Refer to urologist
Asses the chest wall Dehydration and
Pancreatitis Cause minor as spironolactone
for even chest imbalance electrolyte
dehydration and or furosemide
expansion. levels
discomfort
Elevate the head of the Beta and Thick meconium Spironolactone Medical Medical
bed for about 30- 45 glucagon cells blocks bowel (Aldactone) treatment/manage treatment/manage
27
unable to make instead of passing ment ment
insulin through Use to treat swelling
(edema) caused by Assisted In vitro fertilization
Medical treatment liver disease by reproductive (IVF)
Diabetes related
/management Meconium ileus removing excess technology
to CF Adoption and
fluid and improve
Intravenous breathing problems. Semen Analysis surrogacy
Nursing
antipseudomonal Nursing Also, used to treat
Intervention Scrotal Ultrasound
antibiotics like penicillin Intervention conditions which
or cephalosporin NPO body making too Testicular Sperm
Eliminate the pain much aldosterone.
Bricanyl (terbutaline Aspiration
by giving the The infant will be If treated:
sulfate) prescribed pain fed through an
reliever intravenous (IV) Able to bear or
Coamoxiclav have a child
line Furosemide (Lasix)
(amoxicillin with Avoid difficult-to-
clavulanic acid) digest food. Nasogastric tube Take the blood
can also be If not treated:
Steroid inhaler pressure of the client If treated:
Medical inserted in your before giving the
Symbicort Subfertility
treatment/mana child’s nose and medicine. Used to Able to bear or have
Lung function test gement passed into her excrete the sodium a child
stomach to help (𝑁𝑎+) and water
Sputum culture for AFB Fat-soluble remove excess air
vitamin and fluid. If not treated:
Sputum culture supplementation
-Provides extra Infertility
Chest X-RAY Medical
vitamins and
treatment/manage
Mantoux test minerals to the
ment
body.
Lung Transplant Medical
treatment/manag Liver transplant
Pancreatic surgery
HRCT Chest enzyme ement
replacement- It can provide you
Enema
Replaces with a healthier set
endogenous Surgery for of lungs. This can
exocrine complicated allow you to do
pancreatic meconium ileus more activities and
enzyme and aids potentially lengthen
digestion of your life.
starches, fats,
and proteins.
If treated: If not treated:
If treated:
Pancreatic
Effective airway Bowel perforation 28
elastase test: Since that the
clearance <15 μg cirrhosis is not
Peritonitis curable but
If treated: treatable) It would
If not treated:
stop the damage of
Lung failure Enzyme can the liver and
Systematic
reach the prevent
infection
intestine and complications
Death help to digest
foods. Septic shock
If not treated:
If left untreated:
Organ failure Liver cancer
Chronic
pancreatitis
Death
Cystic fibrosis
related to
diabetes

Death
 29

Predisposing factors are factors that put the child at risk for developing a

problem. Inherited two copies of CFTR mutated gene is the predisposing factor.

Precipitating factors are those that trigger the onset illness. Knowledge deficit about

the importance of taking a genetic screening before conception is the precipitating

factor.

During the Pathophysiology of the Cystic Fibrosis there is a missing CFTR-

mediated chloride transport that results to the hyper-absorption of chloride and leads

to abnormal function of the exocrine glands such as mucus glands, digestive glands,

sweat glands. CF primarily affects the function of exocrine glands that makes

substances such as sweat, tears, saliva, milk, and digestive juices, and releases

them through a duct or opening to a body surface.

The respiratory system, digestive system, reproductive system, and

integumentary system are affected by cystic fibrosis disease.

RESPIRATORY SYSTEM

Lungs

With the missing CFTR protein accompanied with the missing inhibitory

effects to the epithelial sodium channel or ENAc, the cells from the bronchial wall will

increase its water and sodium reabsorption, with the process of osmosis the water

cannot diffuse outside the cell particularly the mucus secreting cell to increase

mucus ciliary clearance and airway surface liquid but because of the defective

chloride ion transport the mucus inside the lung will cause to become very thick and

viscous, causing to create an optimal environment for bacteria to grow, resulting to

 30

frequent infections and symptoms such as dyspnea, wheezing, and chest tightness

and further inflammation damaging the bronchial walls. Over time, this damages the

airways and eventually the lung tissue, which can lead to respiratory failure. This

vicious cycle is also why people with cystic fibrosis are more susceptible to

developing other chronic infections including bronchitis, bronchiectasis, and

pneumonia. This mucus blocks the airways, creating lung damage and making it

hard to breathe. Nursing intervention may include by ensuring a well-ventilated room,

monitoring respiration and breast sound, assessing chest wall for even expansion,

and elevating the head if possible for about 30-45 degree.

Treatment for infection included antibiotics such as oral antibiotics,

intravenous and nebulized; the antibiotic used depends on the bacterium present in

the lungs, because some antibiotics are not effective for certain type of bacteria.

Mucolytic and bronchodilator can also be given to maintain a patent airway. If the

condition of the patient is well treated it will alleviate the difficulty of breathing and will

increase the patency of the airways and If not treated the lungs condition will further

deteriorate leading to respiratory failure and death.

DIGESTIVE SYSTEM

Pancreas

The pancreas release enzymes for digestion and produce insulin. About 90

percent of people with CF have sticky mucus that blocks ducts in the pancreas and

prevents enzymes from reaching the small intestine to digest food. Digestive

enzymes from pancreas are amylase (to digest carbohydrates), protease (to digest
 31

protein), lipase (to digest fats), and bicarbonate (to neutralize stomach acid).

Pancreas does not make enough bicarbonate to neutralize the acidity in small

intestine which lead to impaired digestion. Bicarbonate also serves much in the digestive

system. It raises the internal pH of the stomach, after highly acidic digestive juices have finished in

their digestion of food. Bicarbonate also acts to regulate pH in the small intestine. The client will

experience abdominal pain due to the malabsorption. Undigested food in the intestines can

cause pain, cramping, and loss of appetite.

Moreover, when pancreatic enzymes are unable to release it destroys

pancreatic cells and later lead to inflammation and scarring or pancreatitis. The beta

and alpha cells in pancreas also become damage which can cause diabetes.

Nursing Intervention for this scenario are eliminate the pain by giving the prescribed

pain reliever and advice to avoid difficult-to-digest food. For medical

treatment/management, fat-soluble vitamin supplementation that provides extra

vitamins and minerals to the body and pancreatic enzyme replacement that replaces

endogenous exocrine pancreatic enzyme and aids digestion of starches, fats, and

proteins. Pancreatic elastase stool reveal a <15 μg which is clinical significant of

pancreatic insufficiency. If this condition is treated, enzymes can reach the intestine

and help to digest foods. On the other hand, if disease persist, it will cause chronic

pancreatitis and diabetes and leads to death.

Intestine

Cystic fibrosis has historically been considered a pulmonary disease, but with

the increasing life expectancy of these patients, gastrointestinal manifestations are

becoming more important. With the defective chloride ion transport affecting the

pancreases which prevent the release of pancreatic enzyme responsible for the
 32

digestion of macronutrient and the absorption of micronutrient, the patient will

experience malabsorption because of the undigested food secondary to pancreatic

insufficiency , lacking of lipase and the decrease of biliary function to release bile to

emulsify the fats will cause fat malabsorption and it is evident because the patient

with this condition will experience steatorrhea a clinical features of fat malabsorption.

Nursing intervention to the patient may include drinking of plenty of fluid to avoid

dehydration from the increase fluid loss because of malabsorption, reducing dietary

fiber intake and dietary fat intake to control malabsorption. Pancreatic enzyme

replacement can also help the patient to digest food properly improving digestion

and appetite, without proper intervention and treatment patient will manifest

dehydration and discomfort.

Newborn may also experience Meconium ileus and it is often an early sign of

cystic fibrosis. Cystic fibrosis causes intestinal secretions to be abnormally thick and

sticky, and the secretions stick to the lining of the intestine, causing an obstruction of

the small intestine. These extremely sticky secretions are the first indication of illness

in 10 to 20% of children with cystic fibrosis. Newborns with meconium ileus almost

always develop other symptoms of cystic fibrosis later. Nursing intervention may

include NPO to the patient, intravenous feeding, and nasogastric intubation. Medical

management may suggest enema to remove the obstruction and if the obstruction is

severe surgery may be needed, common patient with this condition may experience

complicate meconium ileus. If the condition is not treated it will cause other

complication such as bowel perforation leading to peritoneal infection and systematic

infection, if the infection is systematic it will cause a septic shock to the patient and

organ failure leading to death.

 33

LIVER

Liver makes a fluid called bile that helps the body absorb fat. Bile travels

through small tubes or ducts in the liver and is stored in the gallbladder, which

empties the bile into the small intestine which the important function in digesting fat

and some vitamins. In people with CF, bile gets thick and flows very slowly. So,

Cystic fibrosis can lead to liver disease by causing mucus to build up and block bile

ducts in the liver. This prevents bile from leaving the liver, which causes

inflammation and produces scarring. In severe cases, this scarring can become

permanent, a condition called cirrhosis. As a result, the liver cannot function properly

so the patient turns into Jaundice, Ascites and had an internal bleeding. The nursing

intervention that we are going to conduct to the patient are since the bleeding occur,

monitor the situation of the patient since bleeding is a sign of hypovolemia and run a

test any stools and emesis for blood. Next, since minimize the salt on his diet to

avoid some hyponatremia, take diuretics medicines such as Spironolactone to treat

the edema caused by liver disease and also a furosemide to secretes the water and

sodium but before taking the medication, we need to check the blood pressure if a

person experiencing a hypertension.

Medical treatment or management that can be done is a liver transplant

surgery, it can provide another healthy liver that the client allow to more some

activities and had a potential to lighten his life. If the person is undergo treatment

there's a possibility that would stop the damage of his liver and prevent some further

complications but if it's left untreated it may cause some liver cancer.
 34

REPRODUCTIVE SYSTEM

Male

The CFTR gene mutation that causes vas deferens atrophy develops in early

embryonic development. The lack of the sperm canal, also known as the congenital

bilateral absence of the vas deferens, renders most males infertile (CBAVD). They

are unable to reach and fertilize an egg via intercourse because the sperm never

make it into the semen. One of the causes is hydrocele, a fluid buildup around the

testes that might limit their capacity to operate. Nursing intervention include checking

for tenderness in an enlarged scrotum, assess men during assessment and

screening, and refer to urologist.

Medical management are assisted reproductive technology – refers to

treatments and procedures aimed at achieving pregnancy. These complicated

procedures may be a possibility for patients who have tried a variety of infertility

treatments and still haven't gotten pregnant. Those considering ART should talk to

their doctor about their options, and they may need to see a reproductive specialist.

Semen Analysis - This test is for males who want to check their sperm and sperm

count. This can also be used to assess sperm quality and motility. Scrotal Ultrasound

- utilized to look at a variety of issues involving the scrotum, testicles, or epididymis if

a person suffers an injury, pain, or swelling in or around the testicles, a doctor may

order a testicular ultrasound. Testicular Sperm Aspiration - Testicular sperm

aspiration (TESA) or testicular sperm extraction (TESE) are two methods for

collecting sperm from the testes (TESE). A wide-bore needle is inserted

percutaneous into the testis to acquire TESA samples. If it is treated the patient will

be able to bear or to have a child. And if it not it will remain Infertile.

 35

Female

Due to various faulty cystic fibrosis transmembrane conductance regulator

(CFTR) action, women with cystic fibrosis have thicker cervical mucus. Thicker

mucus can make it more difficult for sperm to access the cervix, lengthening the

time it takes to become pregnant. Poor nutrition can contribute to reproductive

issues by causing irregular ovulation (the release of eggs from the uterus), which

is one of the reasons why some women with CF have difficulty conceiving. Nurse

task is to do further investigation of the vaginal discharge, checking vaginal pH

(You hold a piece of pH paper against the wall of your vagina for a few seconds,

then compare the color of the pH paper to the color on the chart provided with the

test kit, and assess high vaginal swab (HVS). Medical management is In vitro

fertilization (IVF). In IVF, the couple's eggs and sperm are incubated together in a

laboratory to create an embryo. The embryo is then implanted into the woman's

uterus, where it has a chance to implant and result in a successful pregnancy.

Superovulation, Egg Retrieval, Fertilization, and Embryo Transfer are the phases

in IVF. Another is adoption and surrogacy, the birth parents must give formal

approval to the adoption, and their parental rights must be legally terminated after

the infant is born. Legal contracts are made prior to the embryo transfer process in

surrogacy to establish the intended parents as the legal parents of the infant. And

if it is treated the patient will be able to bear or to have a child. And if it not the

patient will Subfertility, a delay in conceiving.

 36

INTEGUMENTARY SYSTEM

Sweat glands

Sweat glands secret primarily salty water using two parallel mechanisms. It causes

changes in the electrolyte transport system causing cells to absorb too much sodium

and water. CF is characterized by problems with the glands that make sweat and

mucus. CF in sweat glands differ from all the organs that are affected by CF. Due to

the missing CFTR protein, chloride ions are not reabsorbed. Differ from other organs

that the chloride ions are hyper absorbed, because of not reabsorbed chloride, the

salt in the sweat increases, and causes excessive sweating. That’s what happened

to client Z, because his sweat test result more than 60mmol/L. Which result to

Hyponatremia a condition where sodium levels in the blood are lower than normal. In

many cases, too much water in the body dilutes the sodium, causing the condition,

and hypochloremia which is an electrolyte imbalance that occurs when there's a low

amount of chloride in your body.

 37

MEDICAL MANAGEMENT

a. Drug Studies

Brand Name Generic MOA Indication Contraindication Dosage Side Nursing

Name Effect/Adverse Consideration
Effects
Creon pancreati Replaces Exocrine Contraindicated Not indicated GI: nausea,  Observe 3
n endogenous pancreatic in patients with diarrhea (with checks and 10
Other: exocrine secretion acute Dosage varies high dosage), rights of
 Pancreaz pancreatic insufficiency; pancreatitis, with condition. bloating. medication
e enzyme and digestive aid in acute Usual initial administration.
 Zenpep aids digestion disease exacerbations of dosage is Other: allergic  To avoid
of starches, associated with chronic 8,000 to reaction, indigestion,
fats, and deficiency of pancreatitis, or 24,000 units perianal monitor client’s
proteins. pancreatic hypersensitivity to of lipase irritation. dietary intake to
enzymes, such drug or pork activity P.O. ensure a proper
as cystic protein or before or with balance of fat,
fibrosis. enzymes. meal or protein, and
snack. Total starch intake.
daily dose can Dosage varies
also be given according to
in divided degree of
doses at 1-2 maldigestion
hour intervals and
throughout the malabsorption,
day. amount of fat in
diet, and
 38

enzyme activity
of individual
preparations.
 Keep in mind
that fewer
bowel
movement and
improved stool
consistency
indicate
effective
therapy.
 Instruct client to
take before or
with meals and
snacks
 Instruct client
not to change
brands without
consulting
doctor.

Brand Generic MOA Indication Contraindication Dosage Side Nursing Consideration

Name Name Effect/Advers
 39

e Effects
Bricany terbutalin Relaxes Bronchospasm  Contraindicat Not indicated CNS:  Observe 3 checks
l e sulfate bronchial in patient with ed in patients nervousness, and 10 rights of
smooth muscle reversible with Dosage varies with tremor, medication
terbutalin by stimulation obstructive hypersensitivi dosage form; drowsiness, administration.
e sulfate beta2- airway ty to drug or dizziness,  Use cautiously in
turbo andrenergenic disease. sympathomim Adults/Children headache, client with CV
haler receptors. Also etic amines 3-12 years of age: weakness disorder,
relaxes uterine  like other hyperthyroidism,
What smooth sympathomim Aerosol inhaler-2 CV: diabetes, or seizure
dosage muscle. etic amines, inhalation palpitations, disorders.
forms it in patients separated by 60 tachycardia,  Inhalator therapy:
comes in: who are seconds interval, arrhythmias, Review instructions
Dry known to repeated q 4 to 6 flushing. for use of inhalator
powder have hours. (included in the
for oral tachyarrhyth GI: vomiting, package).
inhalation: mia’s nausea,  Teach client to
0.5 mg heartburn. perform oral
per dose. inhalation correctly.
Each Respiratory:  Warn client to
inhaler paradoxical discontinue drug
contains bronchospas immediately and
100 m with notify doctor if
doses. prolonged paradoxical
usage, bronchospasm
dyspnea occurs.
 Assess vital signs:
Other: Baseline pulse and
 40

hypokalemia BP and before each

(high doses), dose. If significantly
diaphoresis, altered from
muscle baseline level,
cramps consult physician.
 Most adverse
effects are transient;
however, rapid heart
rate may persist for
a relatively long
time.
 Be aware that
muscle tremor is a
fairly common
adverse effect that
appears to subside
with continued use.
 Adhere to
established dosage
regimen.
 Instruct client to
report as soon as
possible if not
feeling well while
taking Bricanyl
Turbohaler
 41

Brand Generic Name MOA Indication Contraindicatio Dosage Side Effect/Adverse Nursing
Name n Effects Consideration

Augmentin co-amoxiclav An Lower Contraindicated Not CNS: agitation,  Observe 3

(amoxicillin/clavulanat aminopenicillin respiratory in patients with indicated anxiety, insomnia, checks and
Comes e potassium) that prevents infection, hypersensitivity confusion, 10 rights of
with power bacterial cell- otitis to drug or other Adults behavioral changes, medication
for oral wall synthesis media, penicillin’s and and dizziness, administratio
suspensio during sinusitis, in those with a children convulsion. n.
n and replication. skin and previous history weighin  Use
chewable Clavulanic skin of amoxicillin- g 40kg GI: nausea, cautiously in
tablets. acid increases structure associated (88 lb.) vomiting, diarrhea, clients with
amoxicillin infections, cholestatic or over: indigestion, gastritis, other drug
effectiveness and jaundice or stomatitis, glossitis, allergies,
by inactivating urinary hepatic 250mg black hairy tongue, especially to
beta tract dysfunction. P.O. q 8 enterocolitis, cephalospori
lactamases, infections hours or pseudomembranou n’s, and in
which destroy caused by 500mg q s colitis. those with
amoxicillin. susceptibl 12 hours. mononucleos
e strains of For Renal: Interstitial is.
gram- severe nephritis and  Avoid use of
positive infection, hematuria (rare 250-mg
and gram- 500mg q cases). tablet in
negative 8 hours children
organism. or 875mg Hematologic: weighing
P.O. q 12 anemia, below 40kg.
hours. thrombocytopenia, Use
thrombocytopenic chewable
 42

purpura, form instead.

eosinophilia,  Tell client to
leukopenia, take entire
agranulocytosis. quantity of
drug exactly
Other: as
erythematous prescribed,
maculopapular rash, even after
urticarial, feeling
anaphylaxis, better.
overgrowth of  Instruct client
nonsusceptible to take drug
organism, vaginitis. with food to
prevent GI
upset.
 Observe
closely with
large doses
and
prolonged
therapy,
bacterial or
fungal
superinfectio
n may occur.
 Tell client to
call doctor if
a rash
 43

occurs. A
rash is a sign
of an allergic
reaction.

Brand Generic Name MOA Indication Contraindication Dosage Side Nursing

Name Effect/Adverse Consideration
Effects
Steroid Inhaled budesonide: Indicated  Contraindicat Not Same as  Question for
inhaler: corticosteroid/ Inhibits for the ed in patients indicated individual listing hypersensitivity to
LABA accumulation of treatment with for budesonide any
Symbicor combinations: inflammatory cells; of asthma hypersensitivi Adults and formoterol corticosteroids,
t controls rate of in patient ty to and components.
budesonide/for protein synthesis; 6 years of budesonide children CNS: headache  Instruct client to
moterol decreases migration age and and age 12 rinse and gargle
fumarate of older. formoterol and older: Nasal: mild after each use of
dehydrate polymorphonuclear fumarate nasopharyngeal a steroid inhaler
leukocytes (reverses dehydrates. 2 (Two) irritation, to prevent thrush.
capillary inhalation burning,  Watch for
permeability and  Use in twice daily stinging, Candida
lysosomal children (12 hours dryness, cough, infections of the
stabilization at younger than apart) rhinorrhea. mouth or pharynx.
cellular level). 6 years of  In rare cases,
age. Respiratory: inhaled
formoterol Asthma corticosteroids
fumarate  Primary exacerbation, have been linked
dehydrate: bronchitis.
 44

Long-acting treatment of to increased

selective beta2 status GI: nausea, intraocular
agonist that causes asthmaticus vomiting, pressure and
bronchodilation. It or other acute stomach upset, cataract
ultimately increases episodes of diarrhea, development.
cAMP, leading to asthma or dyspepsia. Stop drug if local
relaxation of COPD where irritation occurs.
bronchial smooth intensive Other: back  Instruct patient to
muscle and measures are pain, joint pain, rinse mouth after
inhibition of necessary. dry mouth, loss use, do not
mediator release of taste, swallow rinsed
from mast cells. candidiasis. water.
 Instruct client to
continue use
budesonide and
formoterol as told
by the doctor
even if feeling
well.
 Instruct client to
call provider
immediately if
sign of allergic
reaction occurs.

Brand Generic MOA Indication Contraindication Dosage Side Nursing

Name Name Effect/Adverse Consideration
 45

Effects
AquADEK multivitami Provides AquADEKs is a  Contraindicated Not indicated GI: diarrhea,  Instruct client to
s n extra vitamins complete in patient with constipation, take medicine
and minerals nutritional allergic reaction Age 4-10: stomach upset, as ordered by
Other: to the body. supplement to any single nausea, the doctor.
Vitamax specifically vitamin, 2 tablets daily emesis.  Instruct client
DEKAs designed to multivitamin or or as ordered that the
meet the needs mineral. by physician. Other: muscle medicine can
of cystic fibrosis weakness, be given with or
patients and  Patient taking Over age 10: numbness and without food, if
individuals who anticoagulant tingling stomach upset
have difficulty medication, As directed by occurs, take
absorbing fat- vitamin K physician. with food to
soluble vitamins interferes with reduce stomach
and nutrients the actions of irritation.
anticoagulant  Some products
therapy. may be mixed
with formula,
fruit juice, or
other food or
liquid. Make
sure that the
client knows
how to take and
prepare this
medication.
 Instruct client to
skip missed
 46

dose if it is
close to the
time for the next
dose, and do
not give 2
doses at the
same time.
 Instruct client to
keep the
medicine at
room
temperature in
a dry place
away from
direct light.

Brand Generic MOA Indication Contraindication Dosage Side Nursing

Name Name Effect/Adverse Consideration
Effects
Pulmozym dornase Hydrolyzes To improve  Contraindicated Not indicated EENT:  Observe 3
e alfa DNA in sputum pulmonary in patients pharyngitis, checks and 10
of cystic function and hypersensitive Adults and voice alteration, rights of
fibrosis decrease the to drug or children 5 laryngitis, medication
 47

patients, frequency of products years and conjunctivitis. administration.

causing moderate to derived from the over:  Use a correct
decrease severe Chinese Skin: rash, and approved
viscosity and respiratory hamster ovary 1 ampule urticarial. nebulizers
elasticity of infection in cell. (2.5mg) (sample of
pulmonary patients with inhaled once Other: chest nebulizer are
secretion. cystic fibrosis.  Pulmozyme is daily, use drug pain. listed with
not with an medication
recommended approved leaflet)
for use by nebulizer.  Instruct client to
children under 5 avoid using
years of age. ultrasonic
nebulizer as they
may stop
Pulmozyme from
working properly.
 Be aware that
some client may
benefit from
twice daily
administration
particularly those
over 21 years of
age or with
forced vital
capacity
exceeding 85%.
 Know that drug
 48

is used in
conjunction with
other therapies
for cystic fibrosis.
 Discard cloudy
or discolored
solution.
 Do not mix with
other drugs in
the nebulizer.
Doing so could
lead to a
physical or
chemical
reaction that may
inactivate
dornase alfa.
 Refrigerate drug
in its protective
foil pouch to
protect it from
strong light.
 Instruct client
how to
administer drug
at home
(promote self-
independence).
 49

 Remind client to
breathe only
though the
mouth when
using the
nebulizer. If this
is difficult,
suggest use a
nose clip.
 Tell client that if
coughing occur
during treatment
to turn off the
nebulizer without
spilling drug.
Resume until
coughing
subsides and
turn on nebulizer
and continue
breathing
through the
mouthpiece until
nebulizer cup is
empty or mist is
no longer
produced.
 50

Brand Generic Name MOA Indication Contraindic Dosage Side Effect/Adverse Nursing Consideration
Name ation Effects

Timenti ticarcillin Ticarcillin is Gynecologic Contraindic Not indicated CNS: seizures,  Observe 3 checks
n disodium/clavul extended- infection, ated in neuromuscular and 10 rights of
anate spectrum lower patient with 1 month to excitability, medication
potassium penicillin that respiratory hypersensi 18 years of headache, administration.
inhibits cell-wall tract, urinary tivity to age: giddiness.  Use cautiously in
synthesis during tract bone drug or client with other drug
microorganism joint, and other 80mg- GI: nausea, allergies, especially
replication; skin and skin penicillin’s. 100mg/kg I.V. diarrhea, stomatitis, to cephalosporin’s,
Clavulanic acid structure 30-40 min emesis, epigastric and in those with
increases infection and infusion q 6 pain, flatulence, impaired renal
ticarcillin septicemia to 8 hours pseudomembranou function,
effectiveness by when (maximum s colitis. hemorrhagic
inactivating beta caused by 3.2g) q.i.d conditions,
lactamase, beta- (2wk) CV: phlebitis, vein hypokalemia, or
which destroy lactamase irritation. sodium restriction.
ticarcillin. producing  Before giving ask
strains of EENT: taste and client about allergic
bacteria or Adult: smell disturbance. reaction to penicillin.
by ticarcillin However, not having
susceptible 3.1g-3.2g (3g Metabolic: a history of penicillin
organism. ticarcillin and hypernatremia, allergy is not
100mg hypokalemia. guarantee against a
Clavulanic future allergic
acid) I.V. 30- Skin: Steven- reaction.
40 min Johnson syndrome,  Check CBC and
 51

infusion q 6 pain at injection platelet counts

to 8 hours. site, pruritus, frequently, as
urticaria, rash. ordered. Drug may
cause
Hematologic: thrombocytopenia.
leukopenia,  Monitor serum
neutropenia, potassium, as
eosinophilia, ordered.
thrombocytopenia,  Observe closely.
hemolytic anemia, With large disease
anemia. and prolonged
therapy. Bacterial or
Other: anaphylaxis, fungal superinfection
hypersensitivity may occur,
reaction, especially in elderly,
overgrowth of debilitated, or
nonsusceptible immunosuppressed
organism, edema, client.
fever, chills.  Tell client to report
any adverse effect
reaction promptly.
 Instruct client to alert
nurse if discomfort
occurs at I.V site.
 Advice client to limit
salt intake during
drug therapy
because of high
 52

sodium content.

Brand Generic Name MOA Indication Contraindication Dosage Side Nursing

Name Effect/Adverse Consideration
Effects
Colomyci colistin Colistin is a Colomycin by Contraindicated Not indicated Respiratory:  Instruct client
n (colistimethate cyclic inhalation is also in patient with coughing, to avoid mixing
sodium) polypeptide indicated for the hypersensitivity Nebulized bronchospasm, Colomycin
antibacterial management of to the active Colistin wheezing. with any other
agent adult and substance, products for
belonging to pediatric chronic colistin or to 1 month to 2 EENT: sore nebulization at
the polymyxin pulmonary polymyxin B. years of throat, hoarse the same time.
group. infections due age: voice, taste Some
Polymyxin to Pseudomonas disturbance. medication
work by aeruginosa in 500,000 to 1 may interact
damaging the patients with million units GI: nausea which could
cell membrane cystic fibrosis. vial q 12 stop one of the
and the hours ( b.i.d) Skin: rash medication
resulting from working
physiological 2 to 18  Instruct client
effects are years of to report any
lethal to the age: side effects
bacterium. before having
Polymyxins 1-2 million any further
are selective units vial q dose.
for aerobic 12 hours  Instruct client
Gram-negative (b.i.d) on how to use
bacteria that
 53

have a and prepare

hydrophobic the provided
outer nebulizer and
membrane. Colomycin by
the GP
1. Wash hands
2. Assemble
equipment
3. Check
expiration date
4. Tap Colomycin
vial on surface
to loosen
powder inside.
5. Flip plastic cup
on the vial.
6. Remove foil
seal
7. Remove the
colored bung
from vial.
8. Twist plastic
cap 10ml
liquid ampule,
remove liquid
using 5cc
syringe
9. Put 4cc into
 54

vial of
Colomycin.
10. Throw away
the rest of the
liquid; put the
used syringe
and empty
liquid ampule
into sharp
container.
11. Replace bung
in the
Colomycin vial
and swirl
gently
between
hands to mix,
avoid shaking
as this cause
excessive
frothing
12. Remove bung
and pour liquid
medicine into
the nebulizing
pot.
13. Turn on
compressor
 55

and use
nebulizer.

Brand Generic MOA Indication Contraindication Dosage Side Nursing Consideration

Name Name Effect/Adverse
Effects
Floxape flucloxacillin By binding to Flucloxacillin  Contraindicated Not indicated Common  Observe 3 checks
n specific sodium is in patient with and 10 rights of
penicillin- indicated for hypersensitivity GI: stomach medication
binding the treatment flucloxacillin, Prophylactic upset, diarrhea, administration.
proteins of infections penicillin, any dose nausea,  Warn client about
located inside due to other antibiotic emesis, taking paracetamol
the bacterial sensitive or any of the Primary indigestion, with flucloxacillin.
cell wall, Gram-positive other Prevention dyspepsia. There is a risk of
flucloxacillin organisms, ingredients of blood and fluid
inhibits the including B- the medicine. Birth to 35 EENT: oral abnormality (high
third and last lactamase months: thrush anion gap
stage of producing  Patient having metabolic acidosis)
bacterial cell staphylococci an allergic 125mg twice Other: vaginal which occurs when
wall synthesis and reaction to daily. thrush there is an
which exerts a streptococci. B-lactam increase in plasma
 56

bactericidal Typical antibiotics (e.g. 36 months Uncommon acidity, when

effect upon indication penicillin’s, to 48 flucloxacillin is
many gram includes skin cephalosporin’s months: GI: used concomitantly
positive and soft tissue ) pseudomembra with paracetamol.
organisms infection, 250mg twice nous colitis  Use cautiously with
including B- respiratory  Patient having a daily. client on a
lactamase tract infection, previous history Skin: Erythema controlled sodium
producing and other of flucloxacillin multiforme, diet. Floxapen 250
staphylococci infection associated Secondary urticaria, mg contains 13mg
and cause by jaundice/liver Prevention purpura, sodium and 500mg
streptococci. flucloxacillin dysfunction (Chronic Dermatitis, toxic contain 25mg
sensitive S.aureus epidermal sodium.
organism.  Patient 50 years colonization) necrolysis,  Instruct client to
old or over stevens- take medication
1 month-18 johnson half to one hour
years of syndrome before meals.
age: Medication should
Hematologic: be taken with an
25mg/kg neutropenia, empty stomach.
twice daily or thrombocytopen  Instruct client to
50mg/kg ia, take this
twice daily if medication exactly
necessary as what the doctor
(max 1g told.
twice daily)  Instruct client to
avoid discontinuing
of medication
because some
 57

bacteria may
survive and cause
the infection to
come back.
 Instruct client to
swallow the
capsule whole with
water half to one
hour before meals
to avoid pain in the
esophagus.
Capsule should not
be chewed.
 Instruct client to
take missed
capsule as soon as
remembered, then
the next dose at
the time it is
normally due,
avoid making up
for missed doses
by taking more
than one dose at a
time. This will
increase the
chance of
unwanted side
 58

effects.
 Instruct patent to
report immediately
if side effects
occurs during
treatment of
flucloxacillin
(Floxapen)

b. Laboratory tests

Name of laboratory test: CHEST X-RAY

TEST Result Range value Clinical significance

 59

Chest X- Bilateral hilar lymphadenopathy 5 – 10mm Abnormal: The result indicates that client Z has bilateral
ray enlarge lymph nodes in pulmonary hilar. This abnormality
may cause by infection like TB and pneumonia, silicosis,
and allergies.

CX-ray tests was done in conjunction with taking a medical

history and performing a physical exam to confirm or
exclude a suspected chest since the client is experiencing
a chronic productive cough, wheeze, and difficulty of
breathing which is a sign and symptoms of TB disease.
 
When a client has TB disease in the lungs, the chest x-ray
will likely be abnormal. Infiltrates or cavities may be visible.

Name of laboratory test: MANTOUX TEST

Content Result Range value Clinical significance

MANTOUX Negative 0-4mm Normal: A negative result can be interpreted to mean that the
TEST/ PPD (negative) client has not been infected with the TB bacteria or that the
 60

TEST person has been infected recently and not enough time has
elapsed for the body to react to the skin test.

The Mantoux test is a tool for screening for tuberculosis (TB)

and aid to the physician in the diagnosis of tuberculosis in
client Z’s case.

A positive result means you likely have TB germs in your body.

Name of the laboratory test: T-SPOT test

Text Result Range value Clinical significance

T- Negative Negative: both Normal: A negative result suggests that the infection is
SPOT Panel A-Nil and unlikely.
TEST Panel B-Nil
spot counts are Since Client Z is suspected of having tuberculosis, the test is
≤ 4, including very sensitive and reliable in targeted testing groups and
values <0. provides full testing coverage for him.
The T-SPOT.TB test uses the enzyme-linked immunospot
(ELISPOT) methodology to enumerate M. tuberculosis-
sensitized T cells by capturing interferon-gamma in the vicinity
of T cells.

T cells become sensitized to MTB antigens as part of this

response, and activated effector T cells, both CD4+ and CD8+,
generate the cytokine interferon-gamma in response to these
 61

antigens.
A positive TB test result means only that TB bacteria has been
detected.

Name of the laboratory test: SPUTUM FOR AFB CULTURE

Test Result Range Clinical significance

value
Sputum Negative Negative Normal: A negative result means you likely don't have active
for AFB TB or another mycobacterial infection.
Culture
It indicates that symptoms probably not due to active
mycobacterial infection or there weren't enough bacteria in
the sample for your health care provider to make a diagnosis.

AFB laboratory tests detect microorganisms in client Z's

sputum and aid in identifying an infection caused by AFB, as
well as monitoring therapy effectiveness.

A positive test result from the acid-fast stain confirms

the client has TB and will tell the health worker which
antibiotics should best treat for client Z.

Name of laboratory test: SPUTUM CULTURE

Test Result Range value Clinical significance

 62

Sputum S. aurues Negative Abnormal: the presence of the S. aureus in the sputum
culture indicates that the client have respiratory bacterial
infection. As per evidence that the client have productive
cough and build-up sputum.

Staphylococcus' affinity for cystic fibrosis mucus,

mucociliary problems, and other unknown reasons all
lead to staphylococcus colonization, which causes
progressive lung damage and may influence
Pseudomonas infection.

Name of the laboratory test: HIGH-RESOLUTION CT CHEST

Text Result Range value Clinical significance

HIGH- Bronchiectasis Small airway Abnormal: The bronchioles usually has small airway that
RESOLUTI 5 to 0.3 mm allows the air to come in and out. But bronchiectasis
ON CT causes permanently dilation of bronchi with excessive
CHEST mucus plugging. As a result, there is a reduced airflow. This
condition is commonly caused by TB and pneumonia -
 63

disease affecting the lungs.

This test was done to detect respiratory abnormalities since
Client Z was experiencing a chronic cough, finger clubbing,
wheeze, crackles, and shortness of breath, prompting
further investigations into an underlying chronic respiratory
condition.

Interstitial lung disease, such as pulmonary fibrosis, and

other widespread lung disorders, such as emphysema and
bronchiectasis, are diagnosed and assessed with HRCT.
mucus plugging Thin, watery, Abnormal: A mucus plug is a build-up mucus in the airway
and slippery which causes productive cough and obstruct airflow. This
symptoms can be caused by: infection, GERD, allergies,
bronchiectasis, and cystic fibrosis.

Name of the laboratory test: SWEAT TEST

Test Result Range Clinical significance

value
CHLORI 81 mmol/L 10-35 Too high: The client have a high level of chloride in his sweat
DE milliequiva that indicates he is positive for Cystic Fibrosis.
lents/L A chloride sweat test helps diagnose cystic fibrosis (CF), an
inherited disorder that makes kids sick by disrupting the normal
 64

function of epithelial cells in the lungs.

In cystic fibrosis, the CFTR chloride channel is defective, and

does not allow chloride to be reabsorbed into sweat duct cells.
Consequently, more sodium stays in the duct, and
more chloride remains in the sweat. The concentration
of chloride in sweat is therefore elevated in individuals
with cystic fibrosis.

If the chloride level in your child's sweat is between 30 and 60,

he or she may have metabolic syndrome caused by a defective
cystic fibrosis transmembrane conductance (CFTR) gene.

Name of the laboratory test: GENETIC TESTING

Test Result Range value Clinical significance

Genetic c. 1040G>C p mutation Negative Abnormal: Cystic fibrosis is an autosomal recessive

test exon 14b deletion condition resulting from the pathogenic variants in the CF
transmembrane regulator (CFTR) gene mutation. CF is a
hereditary disease caused by impaired epithelial chloride
 65

channel CFTR function.

The CFTR gene codes for a protein known as Cystic

Fibrosis Transmembrane Conducts Regulator. The
passage of chloride and sodium ions through function
epithelium cells that create saliva, sweat, mucus, tears,
and digestive enzymes is regulated by this protein.

CFTR is a single nucleotide variant type where protein

change occur. The protein change is Arginine 347
Proline.

This mutation causes excessive mucus production and

high chloride in the sweat which is can be seen in client
Z.

Name of the laboratory test: LUNG FUNCTION TEST

Content Result Range value Clinical significance

FVC (forced 73% 80 – 120% Mild obstruction: A low FVC value is a sign of conditions
vital capacity) including: chronic bronchitis, emphysema,
 66

and bronchiectasis, idiopathic pulmonary fibrosis,

scoliosis, and inflammatory lung diseases.
The FVC helps the clinician diagnose a chronic lung
disease, track the progression of the disease through time,
and assess the severity of the condition.

The higher the proportion, the more lung capacity you

have and the healthier your lungs are.

FEV1 (forced 54% >80% Moderate obstruction: The amount of air that can be
expiratory forced to exhale from the lungs is moderate which
volume in 1  indicates client Z is experiencing a breathing obstruction.
s)
EV1 is a measurement of how much mucus is clogging a
person's main airways.
Lower scores indicate that lung damage has progressed.

Significant lung disease is indicated by a FEV1 of less

than 1 L.

Name of the laboratory test: PANCREATIC ELASTASE STOOL TEST

Test Result Range Clinical significance

value
 67

500 - 200 Too low: The result show a too low amount of elastase. It
FECAL <15 μg
μg/g probably means client Z have pancreatic insufficiency. As
ELASTASE
the client has present health history of abdominal pain,
weight loss, and steatorrhea.

Pancreatic enzymes are not release which cause

undigested food.
A higher results indicate that the pancreas is working well.

Chronic pancreatitis, alcohol abuse, pancreatic cancer,

type 1 diabetes, genetic disorders like cystic fibrosis or
Shwachman-Diamond syndrome, inflammation from
digestive diseases like Crohn's disease or celiac disease,
or as a complication from surgery on the pancreas or
nearby parts of the digestive tract are all possible causes of
this damage.

Name of laboratory test: SPUTUM CULTURE

Test Result Range Clinical significance

value
 68

SPUTUM Pseudomonas aeroginosa Negative Not normal: Pseudomonas infections tend to set in

CULTURE after mucoid secretions clog the lung airways which
caused the infection.

Cough, especially cough that produces sputum, is the

most common symptom of bacterial pneumonia and
may indicate a specific disease.

Opportunistic infections of Pseudomonas are a kind

of Pseudomonas infection. This means that the
bacteria only cause infections in people with cystic
fibrosis (CF) or other conditions that weaken the
immune system.

Pseudomonas aeruginosa is one of the most

prevalent bacteria detected in CF clients.
Pseudomonas infects around half of all persons with
CF.

NURSING MANAGEMENT
 69

Name: Client Z Age: 12 Sex: Male

Chief complained: Shortness of breath Admitting physician: Dr. Preni Pansaon Attending physician: Dr. Mikee Siasu
Admitting/final diagnosis: cystic fibrosis Admission: June 8, 2020 at 8:00AM
Assessment Nursing Objective Intervention Rationale Evaluation
diagnosis

Date & time of Ineffective Within 30 1. Establish rapport. 1. To establish effective nurse and Goal met
admission: airway clearance minutes to 2 client interaction.
June 8, 2020 at related to thick hours span of 2. Monitor and 2. To assess the health condition of After 1 hour span of
8:00AM mucus plugging nursing care, record client vital the individual. nursing care, the
as evidenced by client will be able signs. 3. Inhaled β2-adrenergic agonists client was able to
Objective: difficulty of to open airway 3. Administer are first-line therapies for rapid open airway as
Temp: 98.6 F breathing evidenced by medications ( beta- symptomatic improvement of evidenced by
BP: 100/64 secondary to absence of agonist, pulmozyme, bronchoconstriction. These absence of
mmHg cystic fibrosis shortness of salmeterol medications relax smooth muscles shortness of breath.
HR: 78 bpm breath. (Serevent), and and reduce local congestion, reducing
RR: 25 cpm Pediatric nursing Indacterol (Arcapta)) airway spasm, wheezing, and mucus
care plans production
-Shortness of pp589. 4. Administer 4. This treatment opens bronchi and
breath nebulization loosens secretions. It usually causes
-Productive Rationale: (albuterol) as considerable coughing followed by
cough Dysfunctional prescribed 1 before expectoration of mucus and
-wheeze CFTR protein or 2 hour after sometimes vomiting from excessive
gene meals. coughing. Scheduling in relation to
Medications: meals is essential to provide
- terbutaline Impaired maximum benefit of treatment and
sulfate transport of prevent interference with nutrient
 70

- dornase alfa chloride and ingestion. Treatment before breakfast

water in and out helps loosen secretions that built up
of lung cells 5. Collaborate with overnight.
respiratory therapy 5. Help relieve client’s respiratory
excessive mucus regarding as needed distress.
secretion breathing treatments
and deep suctioning
Altered for shortness of
mucociliary breath and
motion ineffective airway
clearance.
Airway 6. Administer
obstruction antibiotic as 6. Administering antibiotics eliminate
prescribed, and causative bacteria of infection that
Ineffective educate client about increases mucus productions. Whilst
airway clearance possible side effects under medication client may
of treatment. experience several side effects.

7. Assess cough for

effectiveness.
7. Habitual cough suppression is
common in clients with cystic fibrosis;
an effective mucus-clearing is
important for adequate airway
8. Monitor clearance.
respiration and 8. To indicative of respiratory distress
breathe sound. and accumulation of mucus. Mucous
plugging will cause an increase in the
respiratory effort to compensate for
 71

airway obstruction. As moving air into

and out of the lungs becomes more
difficult, the breathing pattern alters to
include the use of accessory muscles
9. Encourage and retractions.
increase fluid intake 9. Hydration helps decrease the
to 3000 mL per day viscosity of secretions, facilitating
within cardiac expectoration. Using warm liquids may
tolerance. Provide decrease bronchospasm. Fluids
warm or tepid during meals can increase gastric
liquids. Recommend distension and pressure on the
the intake of fluids diaphragm.
between, instead of
during, meals
10. Assess the
chest wall for even
chest expansion. 10. Unequal chest expansion can
11. Encourage a indicate a pneumothorax– a
frequent and complication of cystic fibrosis.
effective cough- 11. Habitual cough suppression
particularly around results in the retention of mucus;
airway clearance clients should be encouraged to
therapy (ACT). actively and intentionally use effective
12. Monitor oxygen coughing to clear airway mucus.
saturation.
12. Hypoxemia may result from
impaired gas exchange and from the
build-up of secretions and bronchial
 72

13. Assess sputum constriction.

for color, amount, 13. A decreasing amount and
and consistency. frequency of sputum production,
lighter color, and thinner consistency
of sputum indicate improvement in
exacerbation. Scant amounts or
streaking of blood in the sputum can
occur from airway inflammation;
however, major hemoptysis can occur
14. Elevate the head and is a life-threatening emergency.
of the bed for about 14. Promotes better lung expansion.
30-45 degree angle.
15. Perform chest 15. Chest physical therapy helps
physiotherapy on loosen the thick, sticky mucus in the
client, and educate lungs so it can be removed by
client’s family on coughing.
how to perform Chest percussion loosens secretions,
chest physiotherapy and postural drainage facilitates
accurately like chest drainage of secretions so that they
percussion, postural can be expectorated.
drainage for 20-30
minutes. 16. The client is a minor and may
16. Encouraging experience anxiety and fear during the
family support. span of treatment. Family support is
important in relieving client anxiety
and fear.
 73

Assessment Nursing Objectives Interventions Rationale Evaluation

Diagnosis

Date & time of Imbalanced Within 3 days of 1. Establish rapport. 1. To establish effective nurse and June 11, 2020 at
admission: Nutrition: Less my nursing client interaction. 9:00 am
June 8, 2020 at than body care, client will 2. Assess vital signs. 2. To assess the health condition of Goal met
8:00AM requirements exhibit signs of the individual.
related to adequate 3. Administer 3. Client with cystic fibrosis requires After 3 days of my
Objective: inability to digestion and pancreatic enzymes pancreatic enzyme supplementation nursing care, the
Temp: 98.6 F digest nutrients good behaviour with meals and to adequately digest food containing client was able to
BP: 100/64 mmHg and loss of in eating as snacks fat or protein. exhibit signs of
HR: 78 bpm appetite. evidence by a 4. Teach child and 4. To prevent destruction of enteric adequate digestion
RR: 25 cpm weight gain of family appropriate coating that results in inactivation of and good
Weight: 27kg Pediatric 300 grams pancreatic enzyme enzymes and excoriation of oral behaviour in eating
(40.4kg) nursing care administration: mucosa. as evidence by a
plan pp 591 a. Take with meals weight gain of 300
-Poor appetite or snacks b. grams.
-Weight loss Rationale: Capsules can be
-steatorrhea Dysfunctional swallowed whole or Currrent wt:
CFTR protein opened 27.3kg
Medications gene c. Sprinkled on food,
-multivitamins but not crushed or
-pancreatic enzyme Mucus block chewed.
therapy pancreatic duct 5. Administer all fat- 5. Fat-soluble vitamin deficits are
-fat-soluble soluble vitamins with common in cystic fibrosis because of
vitamins Digestive meals and enzymes. fat malabsorption. Vitamin
enzymes supplements must be taken with
(lipase, pancreatic enzymes to be absorbed.
 74

protease, 6. Multidisciplinary input and

amylase) unable 6. Collaborate with a collaboration will optimize therapeutic
to release or registered dietician interventions.
pass in obtaining a full
nutritional
Undigested food evaluation. 7. Malnutrition can be associated with
7. Assess the skin’s an alteration in skin integrity. CF-
Nutrients (fats, color, integrity, and related liver disease can result in
protein, and turgor. jaundice.
carbohydrates) 8. Undertreated malabsorption is
cannot be 8. Assess the common in cystic fibrosis
absorbed in abdomen for
small intestine bloating, fullness,
bowel sounds, or
Weight loss palpable stool mass. 9. CF-related diabetes occurs in up to
9. Monitor for 30% of adolescents and adults with
Malnutrition excessive thirst, CF; glucose intolerance can be
urination, and present intermittently with pulmonary
hunger. Obtain exacerbations.
bedside 10. Chronic constipation can result
blood glucose readin from mucus secretion
gs as ordered. and dehydration in the intestinal
10. Encourage lumen (especially when
liberal hydration and malabsorption is adequately treated
high fibre intake. and stool consistency is normalized).

11. Encourage the 11. Hyponatremic dehydration and

liberal use of salt or salt loss occur easily in the client with
 75

salty food intake. cystic fibrosis as sequelae to CF

defect in the sweat glands.
12. Encourage high 12. To promote optimal digestion.
protein and high-
calorie diet. 13. To assess for potential nutritional
13. Observe problems and determine amount of
frequency and replacement enzymes needed. If
nature of stools stools loose, require more
14. Monitor increase replacement enzyme.
in weight and 14. To be able to determine the
appetite. effectiveness of the treatment.

ASSESSMENT DIAGNOSIS OBJECTIVE INTERVENTION RATIONALE EVALUATION

Objective: Risk for electrolytes Within 8 hours 1. Establish rapport. 1. To establish effective nurse Goal met.
Temp: 98.6 F (hyponatremia) of nursing care, and client interaction.
BP: 100/64 mmHg imbalance related to client will be 2. Monitor and record 2. To assess the health condition After 4 hours
HR: 78 bpm excessive sodium able to maintain client vital signs. of the individual. span of nursing
RR: 25 cpm loss in sweat fluid volume at 3. Administer electrolyte 3. Oral or IV administration of care, client was
Sweat test: secondary to cystic functional level replacements as electrolytes may be prescribed to able to maintain
81mmol/L fibrosis as evidence and free from prescribed. keep electrolyte balance for a normal blood
(normal:10-35) by chloride ion test dehydration. 4. Monitor serum patients at risk for imbalances. sodium level of
of 81mmol/L electrolyte levels. 4. The levels of electrolytes in the 140 mEq/L.
-Poor appetite secondary to cystic body can become too low or too
-Weight loss fibrosis. high. Early detection of
 76

-Steatorrhea abnormality in serum electrolyte

5. Assess for the signs levels allows prompt initiation of
of dehydration including measures to prevent further
NANDA page 893 skin turgor, oral imbalances.
mucosa, etc. 5. This will provide a data that
RATIONALE: 6. Identify any clinical could be used to evaluate the
Mutated CFTR conditions or situations proper intervention that the
protein gene that may be a factor for client need
an imbalance in serum 6. Prevention of electrolyte
Defective ion electrolytes. irregularities begins with the
transport 7. Educate the patient identification of situations that put
about dietary sources of the patient at risk for imbalance.
Chloride and sodium sodium and the use of 7. Patients need to learn to read
is not reabsorb in salt substitutes. labels to identify all sources of
epithelial cells. 8. Encourage the client sodium in foods.
to increase adequate
Hypertonic sweat fluid intake.
8. Water also excreted when
Saltier sweat 9. Monitor patient’s fluid body loses sodium. It also
input and output. prevents skin dryness.
Risk for electrolyte 10. Provide health
imbalance teachings on avoidance
of dehydration. 9. To determine if IV fluid and
electrolyte replacement are
needed
10. To promote awareness on
related factors.
 77

Assessment Nursing diagnosis Objective Intervention Rationale Evaluation

Date & time of Risk for high blood Within 8 hours 1. Establish rapport. 1. To establish effective Goal met
admission: glucose level of my nursing nurse and client
June 8, 2020 at (hyperglycemia) care, client will interaction. After 6 hours of my
8:00AM related to impaired be able to 2. Monitor and record 2. To assess the health nursing care, client
pancreatic insulin maintain normal client vital signs. condition of the was able to maintain
Objective: producing cells blood glucose individual. normal blood glucose
Temp: 98.6 F secondary to cystic level between 3. Assess for signs of 3. Hyperglycemia results level between 80-
BP: 100/64 mmHg fibrosis. 80-140mg/dl hyperglycemia. when there is an 140mg/dl and no
HR: 78 bpm and would not adequate amount of signs and symptoms
RR: 25 cpm Brunner and Sudarth’s manifest signs insulin to glucose. of hyperglycemia.
textbook of medical- of 4. Monitor blood 4. Normal fasting
surgical nursing pp597 hyperglycemia. glucose levels as glucose for adult is 70 to
Stool elastase: <15  fasting and 105mg/dL. Critical value
μg (normal: 500 - Rationale: postprandial levels. of hyperglucemia is
200 μg/g) Dysfunctional CFTR greater than 400mg/dL.
protein gene 5. Assess blood 5. Effective to
glucose level before differentiate the sugar
Impaired transport of meals and bedtime. level before meals and
chloride and water in bedtime. To determine if
and out of pancreatic there is increase and
duct decrease.
 78

6. Administer insulin 6. Insulin aids the

Prevents enzyme from as order. absorption of sugar in
reaching the intestine cells.
7. Educate the client 7. Early recognition is
Enzymes destroy the the signs and crucial. It allows the
pancreatic cells symptoms of client to seek medical
hyperglycemia such assistance immediately.
Impaired beta cells as excessive thirst,
and alpha cells urination, and
hunger. 8. To prevent infection.
Insulin indufficient 8. Instruct client to Client have decrease
clean the foot and sensation in the
Risk for high blood put foot wear. extremities due to
glucose level peripheral neuropathy.
9. The absorption of
9. Instruct client on insulin is fastest in the
proper administration abdomen, followed by
of insulin. the arms and thighs.
Injection of insulin in
same site over time will
result to in lipoatrophy.
10. To maintain overall
10. Educate the blood glucose control.
client on maintaining
the amount of food. 11. Exercise plays a role
11. Educate the in lowering blood
client about the glucose and reduced
benefits and cardiovascular risk
 79

importance of factors.
exercise in the
management of 12. To control high sugar
diabetes. level in blood.
12. Encourage the
client to reduce
eating food high in
sugar.
 80

PROGNOSIS

CRITERIA GOOD FAIR POOR JUSTIFICATION

3 2 1

The client has cough soon after birth

ONSET OF
ILNESS  and persisted the most of his life. He
lost weight at the age of five and
develop a productive cough and
started on asthma treatment 3 years
later. The client also has shortness
of breath and wheeze sound.

The Client hospitalized soon after

DURATION OF
ILLNESS  he returned from china and started
on intravenous antibiotic. He had
asthma treatment years later.

He undergone to 2 weeks treatment

for lower respiratory tract infection.

He was admitted again for 2-week

for Intravenous Antipseudomonal
antibiotics.
Later in his life he was diagnosed
with cystic fibrosis disease after
several investigations.

PRECIPITATIN The possible factors that connects

G
FACTOR  to the client is most likely to be
inherited and triggering factor are
mucus plugging that cause bacterial
infection, sodium chloride loss, and
mal-absorption of nutrients.

There is no engagement to an
unhealthy lifestyle that can trigger
the client’s health condition.

The client has been very

ATTITUDE AND
WILLINGNESS  cooperative in taking his treatment
and medications. Consistent
TO TAKE implementations will surely lead to a
 81

MEDICATION better life.

AND
TREATMENT

FAMILY  The client has family Filipino-

SUPPORT Chinese background and very
supportive on their child's health.
They're giving all their efforts to their
child's condition that includes mental
& emotional support.

Good: 2/5x100=40% Fair: 1/5x100=20% Poor: 2/5x100= 40%

Total: 100%

The highest score rate of the client’s prognosis 40% is for “Good” and “Poor”

categories. The “Poor” category according to the record the client had this fair cough

soon after his birth and developed into productive cough with shortness of breath

and wheeze most of his life. The Client hospitalized soon after he returned from

china and started on intravenous antibiotic. He had asthma treatment years later and

multiple antibiotic treatment. Later in his life his condition was diagnosed with cystic

fibrosis disease after several investigations. He had asthma treatment years later. In

addition, his disease was inherited from both parents.

The score for “Good” category is 40% because the client is very cooperative

on his medications and treatments that will result to a better life if there is no other

conflicts involve, and his supportive parents that had a Filipino-Chinese background

are giving their full support to their child’s condition. The proper implantation and

medications can result to a better way of the client’s life. The condition is easier than

done but it is treatable with proper compliance and strict schedule of medications

and proper treatments this will all be possible with the collaboration of the health

care staff.
 82

The score for “Fair” category is 20% because the possible factors that

connects to the client is most likely to be inherited and triggering factor are mucus

plugging that cause bacterial infection, sodium chloride loss, and mal-absorption of

nutrients. There is no engagement to an unhealthy lifestyle that can trigger the

client’s health condition.

Over all there are total of 100%. Therefore, the overall client’s prognosis of his health

condition is under the “Good” and “Poor” categories.

 83

DISCHARGE PLAN

Medication:

 Encourage the client and the family to adhere to the home medication prescribed

by the doctor.

 Educate the client’s family with the purpose and side effects of each medication.

 Instruct the client and the guardian to take the medicine at exact time with right

dosage.

 Instruct patient to rinse and gargle after each use of a steroid inhaler to prevent

oral thrush.

 Instruct the client and the family to avoid self-medication.

 Instruct the client’s family not to share the medication with anyone.

 Notify the physician immediately in case of severe reaction and side effects

 Advice the client and the family to continue taking colistimethate sodium to treat

bacterial infections.

Exercise:

 Promote breathing exercise.

 Drink plenty of fluid, before, during and after the exercise since during hot

weather, children with CF may lose large amounts of sodium and chloride

through sweat and will need to replace these nutrients.

 Encourage the client to walk or run to improve cardiovascular function

 Encourage the client to avoid high intensity activities and collision sports to

conserve energy and avoid dehydration.

 84

 Encourage the client to swim or try other water exercises to help respiratory

system more efficient.

 Advice the client to do yoga to help client with CF to be more relax and can

breathe easier.

 Advice the client to avoid exercising during hot weather because they lose more

salt when sweating.

 Encourage the client to lift weights to promote muscle strength and bone density.

Treatment:

 Comply with the treatment regimen to assure the health improvement outcome of

the client.

 Encourage to exercise regularly and drink lots of fluids they can help thin the

mucus in the lungs.

 Instruct client to keep updated with any follow-up examinations/therapy

 Discuss to the family the grounds and purposes of treatment to be done to

promote their knowledge.

 Teach the client and the family about the proper administration of medication,

potential side effects of the medication, and the symptoms to ensure client’s

safety.

 Discuss to the client and to the family the dosage, actions, and contraindications

of medication to reduce the risk of complications. Exact dosage and proper timing

is important for the medication to be effective.

 Discuss details in particular for side effects and adverse effects of the medication

to the client. To help the client distinguished if the medication is doing the work
 85

properly. It will also help clients identify the undesired side effects of the

medication that may require intervention.

 Encourage the client to follow special diet to avoid malnutrition

 Encourage the client to talk to the child about the dangers of smoking.

Health Teachings:

 Encourage exercise for breathing.

 Explain the importance of medication compliance.

 Learn how to do chest physical therapy on your child to help keep the child’s

lungs clear of extra mucus.

 Remind your child to wash his or her hands often, and correctly to prevent

infection.

 Teach the child to keep his or her hands away from the face.

 Instruct client to follow the meal plan given.

 Learn about the special dietary needs of your child, the child may need

pancreatic enzymes to help with digestion.

 Eat healthy meals. Maintain a healthy diet; Increase your intake of whole grains,

vegetables, fruits, and protein.

 Advice the client to take nutritional supplements if healthy eating is not enough.

 Walking and swimming are some of the most effective and recommended forms

of exercise for clients with CF.

 Advice the patient to take nutritional supplements if healthy eating is not enough.

 Encourage the parent to always keep the home environment clean, free from

dust to avoid frequent lung infection.

 Instruct the client to avoid eating peanuts

 86

Out-Patient Follow up:

 Instruct the client to return to their attending physician every 2-3months to

achieve maintenance of growth and development, maintenance of as nearly

normal lung function.

 Discuss to the client’s family the importance of regular check-ups for the client’s

health conditions.

DIET

Meal Plan

(1800kcal)

Sunday Calories
Breakfast 1 cup of rice 206
2 boiled eggs 154
1 glass of apple juice 110
Lunch 1 cup of rice 206
1 chicken breast 165
Snack 1 piece siopao asado 330
1 glass mais con yelo 252
Dinner 1 cup of rice 206
1 serving of tuna fish 132
Total 1,761

Monday Calories
Breakfast 1 cup of oats 307
1 glass of orange juice 110
1 avocado 160
Lunch 1 cup of rice 206
1 serving law-uy 240
Snack 1 empanada with chicken 235
breast 150
1 glass of banana shake (no
 87

sugar)
Dinner 1 cup of rice 206
1 serving shrimp 99
Total 1,713

Tuesday Calories
Breakfast 1 cup cereals 307
1 boiled egg 78
1 glass of lychee juice 66
Lunch 1 cup of rice 206
1 fish (escabeche) 350
1 slice of watermelon 30
Snack 1 glass of apple shake (no 109
sugar) 146
1 serving leche flan
Dinner 1 cup rice 206
1 serving of adobong manok 330
(breast part)
Total 1,828

Wednesday Calories
Breakfast 1 cup of rice 206
1 tortang talong 142
1 glass apple juice 110
Lunch 1 cup rice 206
1 serving of vegetable bola- 210
bola
Snack 15g boiled camote 110
1 avocado milkshake (no 241
sugar)
Dinner 1 cup rice 206
1 serving pakbet (no meat) 233
1 banana 110
Total 1,774

Thursday Calories
Breakfast 2 breads 265
1 glass carrot shake 93
 88

10g boiled camote 95

Lunch 1 cup rice 206
1 serving ginisang alugbati 213
Snack 1 siopao asado 330
1 glass orage juice 110
Dinner 1 cup rice 206
1 serving lawuy with shrimp 148
1 banana 110
Total 1,776

Friday Calories
Breakfast 1 cup cereals 307
1 piece apple 95
Lunch 1 cup rice 206
1 ampalaya with egg 273
Snack 1 piece siopao asado 330
1 avocado milkshake 241
Dinner 1 cup rice 206
1 serving tahong 177
Total 1,835

Saturday Calories
Breakfast 1 cup oats 307
1 banana 110
Lunch 1 cup rice 206
1 fish soup 350
Snack 1 piece chicken breast 235
empanada 150
1 glass banana shake
Dinner 1 cup rice 206
1 serving chop soy 142
Total 1,706
 89

EVIDENCE-BASED RESEARCH FINDINGS RELATED TO THE DISEASE

Recent decades have shown a great improvement in survival of individuals

with cystic fibrosis (CF). Registries in Europe, the USA and Canada now

demonstrate that median survival for CF is around 40–50 years (UK 45.1, USA 41.1

and Canada 49.7 years). The median age at death worldwide is around 30 years

because of health inequalities with the dramatic variation in the survival of CF

individuals across Europe. The increased survival can be explained by

implementation of centralized care, newborn screening (NBS) programmes and

evidence-based guidelines including better nutritional care and the introduction of

drugs aimed at the modulation of cystic fibrosis transmembrane conductance

regulator (CFTR) protein. NBS programmes for CF have been widely adopted in

several European countries, Australia, New Zealand and the majority of states in

North America. Major improvements in both nutritional status and lung function in

young children, adolescents and adults as a result of NBS have been reported. Early

diagnosis of CF by NBS and early intervention, including nutritional supplementation

to prevent malnutrition, mucolytic therapy and aggressive treatment of infection with

Pseudomonas aeruginosa, may have contributed to these improvements. Moreover,

nutritional status is positively associated with pulmonary function and survival in

 90

clients with CF. This strongly supports the nutritional strategies and the efforts to

obtain normal growth in CF children, and the importance to maintain adequate

nutritional status in adults with CF. Careful nutritional monitoring as recommended in

European Society of Parenteral and Enteral Nutrition (ESPEN)- ESPHAN-European

Cystic Fibrosis Society (ECFS) guidelines should continue to apply current early

growth recommendation, with goal weight-for-length (WFL) at or above the 50th

percentile on growth charts before age 2. Timely and focused interventions to

improve nutritional status are of great importance to improve prognosis and survival

in CF.

The standard nutritional care for CF has been a high caloric diet with

pancreatic enzyme replacement therapy (PERT) and fat-soluble vitamin

supplementation to achieve an adequate nutritional status. The nutritional status can

also be improved by using CFTR modulators, including correctors and potentiators.

The effect of CFTR potentiation on clinical outcome and subsequently improved

nutritional status is well described by Borowitz et al. (Table 1). A CFTR potentiator,

known as Ivacaftor and commercially available as Kalydeco, has produced results as

respiratory function gain, and pulmonary exacerbation reductions, and several other

achievements, such as better control of diabetes, improvement of pancreatic

function, increase of body weight, improvement of BMI and BMIz scores and quality

of life. Other studies show an improvement in lung function, a slower rate of lung

function decline or no significant changes. With more knowledge about genetic

modifiers of the disease, the individual prognosis can be better defined and

individualized therapy can be optimized.

 91

As survival has increased, the number of adults with CF is expanding, as the

majority of children with CF now live into adulthood. Evidence-based nutritional

guidelines for CF have been developed recently with more attention to adequate

treatment of nutritional-related complications in CF such as CF-related diabetes

(CFRD) and malnutrition.

NUTRITIONAL MANAGEMENT OF CYSTIC FIBROSIS; AN UPDATE FOR

ADEQUATE TREATMENT ACROSS THE LIFESPAN

Recently, excellent evidence-informed and practicebased guidelines on

nutrition care of infants, children and adults with CF have been developed by the

ESPEN-European Society of Paediatric Gastroenterology, Hepatology and Nutrition

(ESPGHAN)-ECFS, and also the Cystic Fibrosis Foundation (CFF) evidence-

informed guidelines on enteral tube feeding (ETF). In addition, the Australian

 92

evidencebased guidelines are expected for October 2017. Also (systematic) reviews

on oral calorie supplements for CF, vitamin D, vitamin E, omega-3 poly unsaturated

acids, probiotics, insulin and oral agents for managing CFRD endorse the

importance of adequate nutritional treatment at different age and disease stages in

CF.

IMPORTANCE OF ADEQUATE GROWTH AND NUTRITIONAL ASSESSMENT

BMI and BMI percentile for age are important measures of nutritional status in

both adults and children with CF. The goal is a WFL of at or above the 50th

percentile in children less than 2 years of age and a BMI of at or above the 50th

percentile for children older than 2 years, meaning that nutritional status is

comparable with that of well-nourished healthy children .The rationale for this goal is

that a positive association exists between pulmonary function, generally measured

by forced expiratory volume in 1 s (FEV1) percentage predicted and nutritional

status. Children with CF who achieve higher WFL at age 2 years have improved

pulmonary and survival outcomes into adulthood. CF care providers should be using

growth charts appropriate to the nationality and ethnicity of the clients. If these are

not available, the WHO growth charts should be used www.who.

Int/childgrowht/standards/en/. Growth and nutritional status should be monitored as

part of routine CF care, including pubertal status. For CF adults over the age of 18

years, the target is a BMI of at or above 22 kg/m2 for women and 23 kg/m2 for men.

Methods and timing of assessment and monitoring of nutritional status in age-related

CF people are well described in the ESPENESPGHAN-ECFS guidelines. In adults,

assessment of body composition is becoming clinically important as depleted or low

fat free mass (FFM) is associated with significant lung disease and impaired

pulmonary function and independent of BMI level. In children, bone mineral content
 93

(BMC) is a more sensitive indicator of nutritional deficit than low BMI; low values of

BMC are correlated with impaired pulmonary function in children with CF.

Furthermore, total body potassium counting is a body composition assessment

method that measures the body cell mass (BCM). The BCM is the metabolically

active component of the FFM and reflects the functional cellular components of the

body involved in biochemical processes and energy metabolism. BCM is not

influenced by hydration. In contrast to the interpretation of total FFM, which can be

affected by hydration changes with growth and disease. Therefore, BCM

measurements are an important reflection of nutritional status in growing children

and those with clinical conditions. However, this method may not be widely available

and other methods such as bioelectrical impedance analysis and dual energy X-ray

absorptiometry can provide information on fat mass and FFM and should be used

where possible. Future research needs to focus on perfecting bedside techniques to

assess body composition, which will assist in improving nutrition-related outcome

measures.

ENERGY BALANCE IN CYSTIC FIBROSIS

Optimal energy intake is critical to the overall health of people with CF.

Insufficient nutritional intake is common in the CF population and is caused by poor

appetite, malabsorption and disturbed body image. This, in combination with

increased caloric expenditure, makes it often difficult to attain an appropriate

nutritional status. The energy balance is not only determined by energy intake (food),

energy expenditure (activity, maintenance and increased demands with

 94

inflammation) and losses (diarrhea and vomiting). In CF, a variety of factors

contribute to individual energy needs, including nutrient maldigestion and/or

malabsorption, presence of pulmonary exacerbation, pulmonary function, FFM, sex,

pubertal status, CFTR mutation, age and other medical complications, such as liver

disease and CFRD. Loss of energy due to malabsorption is a problem in CF clients

with exocrine pancreatic insufficiency. PERT is essential to improve fat

malabsorption in pancreatic insufficient clients. Eightyfive percent to 90% of people

with CF have pancreatic insufficiency, leading to malabsorption of nutrients,

especially fat and fat-soluble vitamins. Addition of proton pump inhibitors may

improve the effectiveness of PERT. Current guidelines recommend lipase intake by

age of the client, by body weight and by grams of fat ingested per day, but a large

variability and inconsistency with new guidelines on nutrition and PERTuse was

found in six European pediatric CF centers. The MyCyFAPP Project has been

started to develop educational and self-management tools for clients’ better

adherence to therapies. Increased caloric expenditure is mainly caused by increased

work of breathing due to chronic lung infection and loss of pulmonary function. In

addition, chronic pulmonary infection and inflammation lead to cytokine-induced

catabolism. In clients with end-stage CF, predication equations for energy needs

underestimate resting energy expenditure (REE). There is a wide variation in the

energy requirements of people with CF. To achieve a normal growth and nutritional

status, energy intake targets for age may need to be increased in children with CF,

although obesity should be avoided. For this reason, recommendations for energy

needs are 110–200% of those required by the healthy population of the same sex

and age. Improvement in weight gain can be achieved using energy dense diets, and

additional oral nutritional supplements (ONS), behavioral interventions, ETF or

 95

parenteral nutrition. A recent systematic review of ONS in three randomized clinical

trials (total of 131 clients) found that ONS did not promote additional weight gain in

moderately malnourished children with CF over and above the use of dietary advice

and monitoring alone. In contrast with children, the use of ONS in adults with CF has

not been adequately studied. Although the Cochrane review concluded that there is

not enough evidence to support the use of ONS, practice-based evidence has shown

that in clinical practice, individually prescribed supplements have increased energy

intake and weight in undernourished patients, but further research should investigate

the long-term effect of supplement use. The CFF recommends the use of ETF in

people with CF who are unable to attain caloric requirements to meet growth/weight

maintenance goals despite evaluation by a multidisciplinary team. Using this

intervention, it is important to monitor for complications such as glucose intolerance

and glucosuria. Gastrostomy tubes are commonly used in patients using ETF, and

jejunostomy tubes are placed in patients with gastroparesis. Although CF is

commonly associated with malnutrition, the proportion of overweight and obese

individuals is increasing. In one US pediatric CF center, 23% of patients with CF

aged 2–18 years were found to have an average BMI percentile greater than 90.

Surprisingly, 88% of the overweight patients and 69% of the obese patients had

CFTR mutations associated with pancreatic insufficiency. In a longitudinal cohort

study that spanned from 1985 to 2011, the proportion of overweight or obese adults

in a Canadian CF center increased from 7 to 18%. The benefit of increasing BMI

greater than 25 in adults seems to be small as improvement in pulmonary function

seems to be blunted. Moreover, recent studies show a higher proportion of obese

individuals with CF having elevated serum triglycerides and total cholesterol.

Furthermore, a cross-sectional study in 32 adults with CF and a reference group of

 96

20 adults without CF showed that a normal-weight BMI with elevated percentage

body fat is associated with reduced lung function. Dietary recommendations should

therefore focus on a balanced diet and a healthy lifestyle with good exercise habits

to achieve an adequate body composition with normal fat and FFM percentages.

SPECIALIZED NUTRITION-RELATED TREATMENTS IN CYSTIC FIBROSIS

For CF patients with pancreatic insufficiency, recent guidelines recommend

the evaluation of plasma levels of fat-soluble vitamins after the start of enzyme and

vitamin supplementation 3–6 months after initiation or change in vitamin therapy; and

annually thereafter. A review of Li et al. compared recent reports of actual dietary

intake and nutritional status of micronutrients such as minerals, trace elements and

vitamins with relevant dietary recommendations for CF. They conclude that although

dietary intake and nutritional status in CF have improved significantly in recent

decades, micronutrient status seems to have diverged. Recommendations for

different age groups differ between countries. The optimal dosages of long-term

micronutrient supplementation require further investigation, so that safety and effect

on reducing lung disease severity and CF-related complications are balanced.

Vitamin D is under investigation for its potential role in gut microbiota modification,

intestinal calcium absorption and bone health, recovery from pulmonary

exacerbations and improvement of lung function. The positive results observed in CF

and non-CF trials of vitamin D supplementation provide a strong rationale for larger,

randomized control trials of long-term, high-dose vitamin D3 supplementation in CF

patients. CF patients usually have abnormal intestinal microbiota because of

exposure to antibiotics. Probiotics could modify the gut microbiota. Several reviews

examined the use of probiotics in the treatment of CF pulmonary exacerbation and

intestinal inflammation. Unfortunately, they lack the scientific quality that is needed
 97

for a recommendation. Until there is more robust evidence supporting the safety of

probiotics in clinical care, they should be used with caution with high-risk patients

such as those with acute exacerbations or those with severe respiratory function.

Also the evidence for dietary supplementation of fatty acids to improve lung function

or anti-inflammatory effects in children or adults with CF is too limited for

recommendations in daily practice. Treatment of CFRD includes education on

diabetes self-management, insulin therapy and aerobic exercise. Although some CF

centers use oral medications to help control diabetes, the CFF clinical practice

guidelines support the use of insulin therapy and this remains the most widely used

treatment method. The achievement of adequate blood glucose levels would be best

to prevent decline in pulmonary function. Patients need to learn to adjust their insulin

dose to the carbohydrate content of the meal, sip feeds or ETF. CFRD patients can

benefit from being seen periodically by a specialized team with expertise in both

diabetes and CF. for CF women planning to become pregnant, a daily supplement of

400 mg of folic acid in the preconception period and throughout the first trimester to

prevent neural tube defects is recommended, similar to non-CF women. A variety of

complications may occur in pregnant CF women, including impaired airway

clearance, chronic respiratory tract infections, diabetes mellitus, pancreatic

insufficiency and nutritional deficiencies. Pregnancy is advised against if the FEV1 is

less than 30% predicted or if pulmonary hypertension is present. Pregnant women

with moderate pulmonary function increase the probability of an optimal outcome.

Management includes adequate nutrition with PERT and fat-soluble vitamins,

management of the chest infections with antibiotics and monitoring of maternal

diabetes. The third trimester may be associated with increasing dyspnea that may

necessitate bed rest with supplemental oxygen and nutritional supplements. Lung
 98

transplantation (LTx) is a well-established treatment option for CF patients with end-

stage lung disease. Ongoing weight loss despite aggressive nutritional

supplementation is one of the criteria for listing for LTx. It is a challenge for dietitians

to optimize energy requirements for improving the nutritional status in pre-LTx

patients with CF. Measurement of REE can be a helpful tool to optimize nutritional

intervention as prediction equations seem to underestimate REE in end-stage CF

patients. After LTx, patients with CF are at risk of developing diabetes, and both

tacrolimus and systemic steroids are known to increase the risk of diabetes post-

transplant.

CONCLUSION

Nutritional care should be closely adapted to the various stages and

complications of CF across the lifespan to extend survival and to improve quality of

life. Moreover, new therapies, such as CFTR modulators and transplantation

techniques, change the needs for nutritional intervention. Recent evidence based

and expert-based guidelines provide up-to date information for optimal nutritional

care of the general patient, but for treatment of individual CF patients personalized

treatment by a specialized CF dietitian is recommended.

 99

REFERENCES

Antibiotic Treatment for cystic fibrosis. (n.d.). CysticFibrosis.

https://www.cysticfibrosis.org.uk/sites/default/files/2020-11/Anitbiotic

%20Treatment.pdf

Bare, B. G. (2003). Brunner and Sudarth’s Medical-Surgical Nursing. 10th edition.

pp. 561. Retrieved June 27, 2021, from https://www.pdfdrive.com

Boyd J.M. (2004). Fertility and pregnancy outcomes in men and women with cystic

fibrosis in the United Kingdom. Retrieved from

https://www.cysticfibrosisjournal.com/article/S1569-1993(19)30839-

2/fulltext#secst0095

Common Cystic Fibrosis Medications. (n.d.). Lurie Children’s. Retrieved June 28,

2021, from https://www.luriechildrens.org/en/specialties-conditions/cystic-

fibrosis-program/ongoing-care-health-maintenance/common-cystic-fibrosis-

medications/

C. (n.d.). Ticarcillin and clavulanic acid. Cystic Fibrosis Medicine. Retrieved June 28,

2021, from https://cysticfibrosis.online/ticarcillin-and-clavulanic-acid/

Chmiel, Aksamit, Chortirmall, Desenbrook, Elborn, Puma, Panganathan, Waters,

Ratjen, J. T. S. E. S. J. S. V. F. (2014, September 11). Antibiotic Management

of Lung Infections in Cystic Fibrosis. I. The Microbiome, Methicillin-Resistant

 100

Staphylococcus aureus, Gram-Negative Bacteria, and Multiple Infections.

NCBI. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5467101/

Colistin: Side Effects, Dosages, Treatment, Interactions, Warnings. (2020, February

5). RxList. https://www.rxlist.com/consumer_coly_mycin_m_colistin/drugs-

condition.htm

Colistimethate: Uses, Interactions, Mechanism of Action | DrugBank Online. (n.d.).

DrugBank. Retrieved June 28, 2021, from

https://go.drugbank.com/drugs/DB01111

Cystic Fibrosis and Exercise. Retrieved June 28, 2021, from

https://cysticfibrosisnewstoday.com/cystic-fibrosis-and-exercise/

Discharge Instructions for Cystic Fibrosis. Retrieved June 29, 2021, from

https://www.fairview.org/PatientEducation/Articles/English/d/i/s/c/h/Discharge

Instructions_for_Cystic_Fibrosis_86325

Hollander, Heijerman, Roos, F. H. N. (2017, November). The optimal approach to

nutrition and cystic fibrosis: Latest evidence and recommendations.

ResearchGate.

https://www.researchgate.net/publication/320433159_The_optimal_approach

_to_nutrition_and_cystic_fibrosis_Latest_evidence_and_recommendations

How Pulmozyme works to fight cystic fibrosis. (n.d.). Pulmozyme. Retrieved June 28,

2021, from https://www.pulmozyme.com/patient/about-pulmozyme/how-it-

works.html

Kochhar, S. (2016).Treating vaginal discharge. London, SE24 0PB.Retrieve from:

 101

https://www.independentnurse.co.uk/clinical-article/treating-vaginal

discharge/143597/

Lippincott Williams & Wilkins Company. (n.d.). Nursing2012 Drug Handbook, 32nd

Edition (Nursing Drug Handbook). Library Genesis. Retrieved June 28, 2021,

from http://libgen.li/ads.php?md5=2317aed8db533114e349568753694e1a

Martin, P. (2014, April 19). 5 Cystic Fibrosis Nursing Care Plans. Nurselabs.

https://nurseslabs.com/cystic-fibrosis-nursing-care-plans/5/

OTC Product News. (2007, March 1). Pharmacy Times.

https://www.pharmacytimes.com/view/2007-03-6331

Ong T, Marshall SG, Karczewski BA, et al. (2021). Cystic Fibrosis and Congenital

Absence of the Vas Deferens. Retrieved from

http://www.ncbi.nlm.nih.gov/books/NBK1250/.

Pool, J. (2019). How Does Cystic Fibrosis Affect Reproductive Health and Fertility?

Retrieve from: https://cystic-fibrosis.com/infertility

Quinton, P. (2007). Cystic fibrosis: lessons from the sweat gland. Retrieved from

https://pubmed.ncbi.nlm.nih.gov/17557942/.

Recommendations | Cystic fibrosis: diagnosis and management | Guidance | NICE.

(2017, October 25). NICE.

https://www.nice.org.uk/guidance/ng78/chapter/recommendations#:%7E:text=
 102

Ren CL, Morgan RL, Oermann C, et al. (n.d). Cystic Fibrosis Foundation Pulmonary

Guidelines: Use of Cystic Fibrosis Transmembrane Conductance Regulator

Modulator Therapy in Patients with Cystic Fibrosis. From:

https://pubmed.ncbi.nlm.nih.gov/29342367/.

Sabharwal, S. (2016). Gastrointestinal Manifestations of Cystic Fibrosis. Retrieved

from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4865785/

Simon, Mallory, Hoppin, R. G. A. (n.d.). Cystic fibrosis: Antibiotic therapy for chronic

pulmonary infection. UptoDate. https://www.uptodate.com/contents/cystic-

fibrosis-antibiotic-therapy-for-chronic-pulmonary-infection

Symbicort (budesonide and formoterol) for the Treatment of Asthma. (2019,

November 20). Clinical Trials Arena.

https://www.clinicaltrialsarena.com/projects/symbicort-budesonide-and-

formoterol-for-the-treatment-of-asthma/

Swearingen, P. L. (2003, August 5). All-in-One Nursing Care Planning Resource:

Medical-Surgical, Pediatric, Maternity, and Psychiatric-.

https://www.Pdfdrive.Com. https://www.pdfdrive.com/all-in-one-nursing-care-

planning-resource-medical-surgical-pediatric-maternity-and-psychiatric-

mental-health-e175333117.html

Welson, R B. (2020). Home and natural remedies for upset stomach. Medical News

Today. Retrieved from:

https://www.medicalnewstoday.com/articles/322047#Twenty-one-home-

remedies

(2016). Fertility in Women with CF. Cystic Fibrosis foundation. Retrieved from:
 103

https://www.cff.org/Life-With-CF/Transitions/Reproductive-Health-and

Fertility/CF-and-the-Female-Reproductive-System/Fertility-in-Women-WithCF/

(2016). Cystic Fibrosis and the Digestive System. How does CF affect the digestive

system? Health Encyclopedia. Retrieved from:

https://www.urmc.rochester.edu/encyclopedia/content.aspx?

ContentTypeID=90&ContentID=P02934

(2016). Fertility in Men with CF. Cystic Fibrosis Foundation. Retrieved from:

https://www.cff.org/Life-With-CF/Transitions/Reproductive-Health-and-

Fertility/CF-and-the-Male-Reproductive-System/Fertility-in-Men-With-CF/

(2017).Could the environment be having an effect on cystic fibrosis? Cystic Fibrosis

Trust. Retrieved from: https://www.cysticfibrosis.org.uk/news/environmentand-

cf

(2017). Digestive system and cystic fibrosis. Cystic Fibrosis Trust. Retrieved from:

https://www.cysticfibrosis.org.uk/what-is-cystic-fibrosis/how-does-cystic

fibrosis-affect-the-body/cystic-fibrosis-complications/digestive-system

(2017).The Digestive Tract. Cystic Fibrosis Foundation. Retrieved from:

https://www.cff.org/Life-With-CF/Daily-Life/Fitness-and-

Nutrition/Nutrition/Taking-Care-of-Your-Digestive-System/The-Digestive-Tract/
 104

(2017). Additional symptoms of cystic fibrosis. Cystic Fibrosis Trust. Retrieved from:

https://www.cysticfibrosis.org.uk/what-is-cystic-fibrosis/how-does-cystic

fibrosis-affect-the-body/symptoms-of-cystic-fibrosis/additional-complications

(2019). Cirrhosis. Mayo Clinic. Retrieved from:

https://www.mayoclinic.org/diseases

conditions/cirrhosis/diagnosis-treatment/drc-20351492

(2019). Cystic Fibrosis & the Liver. Center for Liver Disease and Transplantation.

Retrieved from: https://columbiasurgery.org/conditions-and-treatments/cystic-

fibrosis-liver

(2019). Fertility and cystic fibrosis. Retrieved June 30, 2021,

from:

https://www.cysticfibrosis.org.uk/what-is-cystic-fibrosis/how-does-cystic

fibrosis-affect-the-body/symptoms-of-cystic-fibrosis/fertility

(2020). What Is Distal Intestinal Obstructive Syndrome (DIOS)? Cystic-Fibrosis.com.

Retrieved from: https://cystic-fibrosis.com/clinical/dios

(2020). Learn About Cystic Fibrosis. American Lung Association Scientific and

Medical
 105

Editorial Review Panel. Retrieved from: https://www.lung.org/lung-health-

diseases/lung-disease-lookup/cystic-fibrosis/learn-about-cystic-fibrosis

Cne, F. R. P. M. H. M., Faanp, K. J. D. M. R. A. F., Cne, R. D. R. M. M. C. O., Faan,

H. D. P. R. A. C. C. A., & Fnp-Bc, R. C. R. D. (2019). Lewis’s Medical-

Surgical Nursing: Assessment and Management of Clinical Problems, Single

Volume (11th ed.). Mosby.