University of Cebu – Banilad

College of Nursing
Cebu City

A Resource Unit on

COPD: CYSTIC
FIBROSIS
Submitted by:

BSN 4 – L Group 35:

Magalona, Roselle B.
Milallos, Mark Gilbert S.
Mondido, Ryan Anthony P.
Monilar, Mary Grace B.
Munda, Angela May O.
 Submitted to:

Ms. Ramelda G. Villarazo, RN

Clinical Instructor
University of Cebu – Banilad
College of Nursing
Cebu City

A Resource Unit on
CYSTIC FIBROSIS

GENERAL OBJECTIVES:
After 90 minuteS of lecture – discussion the BSN $ - L Group 35 students will be able to acquire adequate knowledge, gain competent
skills and develop positive attitudes on the concept CYSTIC FIBROSIS.

SPECIFIC CONTENTS METHODOL T.A RESOUR EVALUATI

OBEJECTIVES OGY CES ON
After 90 minutes
of lecture
discussion the INTRODUCTION
BSN 4 – L Group
35 students will Cystic fibrosis (also known as CF or mucoviscidosis) is a common
be able to: hereditary disease which affects the entire body, causing progressive
disability and often, early death. The name cystic fibrosis refers to the
1. Discuss the characteristic scarring (fibrosis) and cyst hairy within the pancreas, first
overview of recognized in the 1930s. Difficulty breathing is the most serious symptom
Cystic Fibrosis. and results from frequent lung infections that are treated, though not
cured, by antibiotics and other medications. A multitude of other
symptoms, including sinus infections, poor growth, diarrhea, and
infertility result from the effects of CF on other parts of the body.
CF is caused by a mutation in a gene called the cystic fibrosis
transmembrane conductance regulator (CFTR). This gene helps create
sweat, digestive juices, and mucus. Although most people without CF
have two working copies of the CFTR gene, only one is needed to prevent
cystic fibrosis. CF develops when neither gene works normally. Therefore,
CF is considered an autosomal recessive disease.
 CF is most common among Caucasians and Ashkenazi Jews; one
in 25 people of European descent carry one gene for CF. Approximately
30,000 Americans have CF, making it one of the most common life-
shortening inherited diseases. Individuals with cystic fibrosis can be
diagnosed prior to birth by genetic testing or in early childhood by a
sweat test. There is no cure for CF, and most individuals with cystic
fibrosis die young — many in their 20s and 30s from lung failure.
Ultimately, lung transplantation is often necessary as CF
Cystic fibrosis is a genetic disorder that affects the digestive system
and lungs. It causes children to be more likely to have infections in their
lungs repeatedly. This happens because there is a disruption in the
normal function of the body’s epithelial cells. These cells line
passageways in the liver, lungs, pancreas, reproductive, and digestive
systems. The skin’s sweat glands are also made up of epithelial cells.
Patients with cystic fibrosis continuously have an excessive buildup of
mucus in the lungs and passageways. Cystic fibrosis poses a serious
threat to patients because of the excessive mucus buildup in the lungs.
The mucus is very thick and can create the perfect environment for
bacteria to develop and grow, especially pseudomonas aeruginosa, which
can cause severe life-threatening infections or nosocomial infections in
persons with cystic fibrosis. A cystic fibrosis sufferer has defective CFTRs
(cell membranes), and their immune system is unable to offer proper
immune response to fight such infections. Therefore, these infections can
be deadly.
A baby with cystic fibrosis usually gets this infection by the age of
3. By the age of 10, the infection has become chronic in many patients.
Many cystic fibrosis sufferers do not live to be in their 40s.
Another danger of cystic fibrosis is maldigestion due to a poor-
working pancreas. This can lead to symptoms of steatorrhea and
malabsorption. The stools will become greasy and large, and may even
have visual droplets of fat. With this condition, the patient might have a
2. Define related difficult time gaining weight.
terms. People who have CF produce a defective version of a protein called
CFTR (Cystic Fibrosis Transmembrane Conductance Regulator). This is
responsible for transport of salts and water across cell membranes. This
means that in certain parts of the body, the secretions lack water,
becoming sticky.
 DEFINITION OF TERMS

1. Epithelial cells – cells that cover the surface of the body and line its
cavities. Epithelial cells help to protect or enclose organs; some produce
mucus or other secretions. Certain types of epithelial cells have tiny hairs
called cilia, which help remove foreign substances
2. Fibrous Tissue – Tissue composed of bundles of collagenous white
fibers between which are rows of connective tissue cells. It is simply
termed as scar.
3. Mucus – is a slippery secretion produced by, and covering, mucous
membranes. It is a viscous colloid containing antiseptic enzymes (such as
lysozyme) and immunoglobulins that serves to protect epithelial cells in
the respiratory, gastrointestinal, urogenital, visual, and auditory systems
in mammals; the epidermis in amphibians; and the gills in fish. Mucus
also contains mucins, produced by goblet cells in the mucous membranes
and submucosal glands, and inorganic salts suspended in water. The
average human body produces about a liter of mucus per day.
3. Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)
– is a cAMP-regulated chloride channel that resides in the apical
membrane of many epithelial cells. Channel opening requires
phosophorylation of serine residues in an intracellular regulatory domain
by protein kinase A and as the binding and hydrolysis of ATP by
intracellular nucleotide binding domains. Besides conducting the chloride
ion, CFTR also regulates the function of other membrane proteins, directly
or indirectly, notably the outwardly rectifying chloride channel and the
epithelial sodium channel. The disease cystic fibrosis is caused by
mutations in CFTR, which can result in defective protein production,
defective processing and degradation in the endoplasmic reticulum, or
defective channel pore properties or gating properties.
4. Pseudomonas aeruginosa – is a common bacterium which can
cause disease in animals and humans. It is found in soil, water, skin flora
3. Review the and most man-made environments throughout the world. It thrives not
Anatomy and only in normal atmospheres, but also with little oxygen, and has thus
Physiology of the colonized many natural and artificial environments. It uses a wide range
Respiratory of organic material for food; in animals, the versatility enables the
System. organism to infect damaged tissues or people with reduced immunity.
The symptoms of such infections are generalized inflammation and
sepsis. If such colonization’s occur in critical body organs such as the
lungs, the urinary tract, and kidneys, the results can be fatal.
5. Nosocomial Infection – are infections which are a result of treatment
in a hospital or a healthcare service unit, but not secondary to the
patient’s original condition. Infections are considered nosocomial if they
first appear 48 hours or more after hospital admission or within 30 days
after discharge.

THE ANATOMY AND PHYSIOLOGY OF THE RESPIRATORY SYSTEm

FUNCTIONS:
1. Gas exchange. The respiratory system allows oxygen from the air
to enter the blood and carbon dioxide to leave the blood and enter
the air.
2. Regulation of blood pH. The respiratory system can alter blood pH
by changing blood carbon dioxide levels.
3. Voice production. Air movement past the vocal cords makes sound
and speech possible.
4. Olfaction. The sensation of smell occurs when airborne molecules
are drawn into the nasal cavity.
5. Innate immunity. The respiratory system provides protection
against some microorganisms by preventing their entry into the
body and by removing them from respiratory surfaces.
UPPER RESPIRATORY TRACT:

Nose
The nose consists of the external nose and the nasal cavity. The
external nose is the visible structure that forms a prominent feature of
the face. Most of the external nose is composed of hyaline cartilage. The
nasal cavity extends from the nares to the choane. The nares or nostrils
are the external openings of the nose, and the choane are the openings
into the pharynx. The nasal septum is a partition dividing the nasal cavity
into right and left parts.
Paranasal sinuses are air-filled spaces within bone. The maxillary, frontal,
ethmoidal, and sphenoidal sinuses are named after the bones in which
they are located. They reduce the weight of the skull, produce mucus,
and influence the quality of the voice by acting as resonating chambers.
The nasolacrimal ducts, which carry tears from the eyes, also open into
the nasal cavity.

Pharynx
 The pharynx is the common passageway of both the respiratory
and digestive systems. The nasopharynx is the superior part off the
pharynx. It is located posterior to the choanae and superior to the soft
palate. The uvula is the posterior extension of the soft palate. The
posterior part of the nasopharynx contains the pharyngeal tonsil.
The oropharynx extends from the uvula to the epiglottis, and the oral
cavity opens into the oropharynx. The palatine tonsils are located in the
lateral walls near the border of the oral cavity and the oropharynx. The
lingual tonsil is located on the surface of the posterior part of the tongue.
The laryngopharynx passes posterior to the larynx and extends from the
tip of the epiglottis to the esophagus.

LOWER RESPIRATORY TRACT

Larynx
The larynx is located in the anterior throat, and it is continuously
superiorly with the pharynx and inferiorly with the trachea. The larynx
consists of an outer casing of nine cartilages that are connected to one
another by muscles and ligaments. Three of the nine cartilages are
unpaired, and six of them form three pairs. The largest cartilage is the
unpaired thyroid cartilage, or the Adam’s apple. The most inferior
cartilage of the larynx is the unpaired cricoid cartilage. The third unpaired
cartilage is the epiglottis. The epiglottis helps prevent swallowed
materials from entering the larynx. The six paired cartilages consist of
three cartilages on either side of the posterior part of the larynx. The top
cartilage on each side is the cuneiform cartilage, the middle cartilage is
the corniculate cartilage, and the bottom cartilage is the arytenoid
cartilage.
Two pairs of ligaments extend from the posterior surface of the thyroid
cartilage to the paired cartilages. The superior pair forms the vestibular
folds, or false vocal cords, and the inferior pair composes the vocal folds,
or true vocal cords.

4. Trace the Trachea

Pathophysiology The trachea, or windpipe, is a membranous tube that consists of
of the disease connective tissue and smooth muscle, reinforced with 16-20 C-shaped
process. pieces of cartilage.
Refer to appendix
1 for the Bronchi
diagram. The trachea divides into the left and right main (primary) bronchi,
each of which connects to a lung. The left main bronchus is more
horizontal than the right main bronchus because it is displaced by the
heart. The right main bronchus is more vertical and is in direct line with
the trachea.

Lungs
The lungs are contained within the thoracic cavity. Each lung is
surrounded by a separate pleural cavity. Each pleural cavity is lined with
a serous membrane called the pleura. The pleura consist of parietal and
visceral pleura. The parietal pleura, which lines the walls of the thorax,
diaphragm, and mediastinum, is continuous with the visceral pleura,
which covers the surface of the lung. Between the parietal and visceral
pleurae is filled with a small volume of pleural fluid produced by the
pleural membranes.
The lungs are the principal organs of respiration. Each lung is cone-
shaped, with its base resting on the diaphragm and its apex extending
superiorly to a point about 2.5 cm above the clavicle. The right lung has
three lobes called the superior, middle, and inferior lobes. The left lung
has two lobes called the superior and inferior lobes. Each lobe is divided
into bronchopulmonary segments separated from each other by
connective tissue septa. There are 9 bronchopulmonary segments in the
left lung and 10 in the right lung.
The main bronchi branch many times to form the tracheobronchial
tree. Each main bronchus divides into lobar bronchi as they enter their
respective lung. The lobar (secondary) bronchi, two in the left lung and
three in the right lung, conduct air to each lobe. The lobar bronchi in turn
give rise to segmental (tertiary) bronchi, which extend to the
bronchopulmonary segments of the lungs. The bronxhi continue to
branch many times, finally giving rise to bronchioles. The bronchioles also
subdivide numerous times to give rise to terminal bronchioles, which then
subdivide into respiratory bronchioles. Each respiratory bronchiole
subdivides to form alveolar ducts and open into alveoli, which are small
air sacs. The alveolar ducts end as two or three alveolar sacs, which are
chambers connected to two or more alveoli. There are about 300 million
alveoli in the lungs.
The lungs have two lymphatic supplies. The superficial lymphatic
 vessels are deep to the visceral pleura and function to drain lymph from
the superficial lung tissue and the visceral pleura. The deep lymphatic
vessels follow the bronchi and function to drain lymph from the bronchi
and associated connective tissues.

THE PATHOPHYSIOLOGY OF CYCTIC FIBROSIS IN THE

RESPIRATORY SYSTEM
5. Know the signs
and symptoms of There are several mechanisms by which mutations cause problems
the disease with the CFTR protein. ΔF508, for instance, creates a protein that does
specifically in the not fold normally and is degraded by the cell. Several mutations, which
Respiratory are common in the Ashkenazi Jewish population, result in proteins that
System. are too short because production is ended prematurely. Less common
mutations produce proteins that do not use energy normally, do not allow
chloride to cross the membrane appropriately, or are degraded at a faster
rate than normal. Mutations may also lead to fewer copies of the CFTR
protein being produced.
The protein created by this gene is anchored to the outer
membrane of cells in the sweat glands, lungs, pancreas, and other
affected organs. The protein spans this membrane and acts as a channel
connecting the inner part of the cell (cytoplasm) to the surrounding fluid.
This channel is primarily responsible for controlling the movement of
chloride from inside to outside of the cell; however, in the sweat ducts it
facilitates the movement of chloride from the sweat into the cytoplasm.
When the CFTR protein does not work, chloride is trapped inside the cells
in the airway and outside in the skin. Because chloride is negatively
charged, positively charged cations cross into the cell because they are
affected by the electrical attraction of the chloride ions. Sodium is the
most common ion in the extracellular space and the combination of
sodium and chloride creates the salt, which is lost in high amounts in the
sweat of individuals with CF. This lost salt forms the basis for the sweat
test.
How this malfunction of cells in cystic fibrosis causes the clinical
manifestations of CF is not well understood. One theory suggests that the
lack of chloride exodus through the CFTR protein leads to the
accumulation of more viscous, nutrient-rich mucus in the lungs that
allows bacteria to hide from the body's immune system. Another theory
proposes that the CFTR protein failure leads to a paradoxical increase in
 sodium and chloride uptake, which, by leading to increased water
reabsorption, creates dehydrated and thick mucus. Yet another theory
focuses on abnormal chloride movement out of the cell, which also leads
to dehydration of mucus, pancreatic secretions, biliary secretions, etc.
These theories all support the observation that the majority of the
damage in CF is due to blockage of the narrow passages of affected
organs with thickened secretions. These blockages lead to remodeling
and infection in the lung, damage by accumulated digestive enzymes in
6. Understand the pancreas, blockage of the intestines by thick faeces, etc.
and know the The lungs of individuals with cystic fibrosis are colonized and
Medical infected by bacteria from an early age. These bacteria, which often
Management of spread amongst individuals with CF, thrive in the altered mucus, which
the disease. collects in the small airways of the lungs. This mucus leads to the
formation of bacterial microenvironments known as biofilms that are
difficult for immune cells and antibiotics to penetrate. Viscous secretions
and persistent respiratory infections repeatedly damage the lung by
gradually remodeling the airways which makes infection even more
difficult to eradicate.
Over time, both the types of bacteria and their individual
characteristics change in individuals with CF. In the initial stage, common
bacteria such as Staphylococcus aureus and Hemophilus influenzae
colonize and infect the lungs. Eventually, however, Pseudomonas
aeruginosa (and sometimes Burkholderia cepacia) dominates. Once
within the lungs, these bacteria adapt to the environment and develop
resistance to commonly used antibiotics. Pseudomonas can develop
special characteristics that allow the formation of large colonies, known
as "mucoid" Pseudomonas, which are rarely seen in people that do not
have CF.

SYMPTOMATOLOGY

The hallmarks of cystic fibrosis are salty tasting skin, normal

appetite but poor growth and poor weight gain, excess mucus production,
frequent chest infections and coughing/shortness of breath. Males can be
infertile due to congenital absence of the vas deferens. CF symptoms
often appear in infancy and childhood, with meconium ileus being a
typical finding in newborn babies. As the child grows, he or she will have
to exercise to release mucus stuck to the alveoli. Cilial epithilial cells in
the patient have a mutated protein that instead of creating the right resin
that is used to prevent the alveoli from collapsing, it makes a thicker
resin, mucus. Poor growth is a hallmark of CF. Children with CF typically
do not gain weight or height at the same rate as their peers, and
occasionally are not diagnosed until investigation is initiated for poor
growth. The causes of growth failure are multi–factorial and include
chronic lung infection, poor absorption of nutrients through the
gastrointestinal tract, and increased metabolic demand due to chronic
illness.
Lung disease results from clogging the airways due to mucosa
build-up and resulting inflammation. Inflammation and infection will cause
injury and structural changes to the lungs, leading to a variety of
symptoms. In the early stages, incessant coughing, copious phlegm
production, and decreased ability to exercise are common. Many of these
symptoms occur when bacteria that normally inhabit the thick mucus
grow out of control and cause pneumonia. In later stages of CF, changes
in the architecture of the lung further exacerbate chronic difficulties in
breathing. Other symptoms include coughing up blood (hemoptysis),
changes in the major airways in the lungs (bronchiectasis), high blood
pressure in the lung (pulmonary hypertension), heart failure, difficulties
getting enough oxygen to the body (hypoxia), and respiratory failure
requiring support with breathing masks such as bilevel positive airway
pressure machines or ventilators. In addition to typical bacterial
infections, people with CF more commonly develop other types of lung
disease. Among these is allergic bronchopulmonary aspergillosis, in which
the body's response to the common fungus Aspergillus fumigatus causes
worsening of breathing problems. Another is infection with
Mycobacterium avium complex (MAC), a group of bacteria related to
tuberculosis, which can cause further lung damage and does not respond
to common antibiotics.
Mucus in the paranasal sinuses is equally thick and may also cause
blockage of the sinus passages, leading to infection. This may cause
facial pain, fever, nasal drainage, and headaches. Individuals with CF may
develop overgrowth of the nasal tissue (nasal polyps) due to
inflammation from chronic sinus infections. These polyps can block the
nasal passages and increase breathing difficulties.

GENERAL MEDICAL MANAGEMENT

Pharmacologic Interventions:
1. Antimicrobial therapy as indicated for pulmonary infection.
o Oral or I.V. antibiotics as required.
o Inhaled antibiotics, such as gentamicin or tobramycin,
may be used for severe lung disease or colonization of
organisms.
2. Bronchodilators to increase airway size and assist in mucus
clearance.
3. Pulmozyme recombinant human DNase (an enzyme)
administered via nebulization to decrease viscosity of secretions.
4. Pancreatic enzyme supplements with each feeding.
o Favored preparation is pancrelipase.
o Occasionally, antacid is helpful to improve tolerance of
enzymes.
o Favorable response to enzymes is based on tolerance of
fatty foods, decreased stool frequency, absence of
steatorrhea, improved appetite, and lack of abdominal pain.
5. Gene therapy, in which recombinant DNA containing a
corrected gene sequence is introduced into the diseased lung
tissue by nebulization, is in clinical trials.

SURGERY
Surgery is risky in people with severe COPD. Postoperatively, many
normal patients temporarily have reduced lung volumes, rapid shallow
breathing, and an impaired ability to take in oxygen and expel carbon
dioxide. These routine postoperative problems add additional stress to the
already compromised respiratory systems of patients with COPD. One
result is that patients with severe COPD develop postoperative pneumonia
13 times more often than patients with normal lung function.
(Preoperative antibiotics can reduce the high rate of postoperative
pneumonia.)
Nonetheless, the lack of alternative treatments for severe COPD has
led to the development of three surgical procedures that attempt to
improve and prolong the lives of COPD patients. The techniques are lung
transplantation, lung volume reduction surgery, and bullectomy (Gold,
2005a).
Lung Transplanatation
Lung transplantation often becomes necessary for individuals with
cystic fibrosis as lung function and exercise tolerance declines. Although
single lung transplantation is possible in other diseases, individuals with
CF must have both lungs replaced because the remaining lung might
contain bacteria that could infect the transplanted lung. A pancreatic or
liver transplant may be performed at the same time in order to alleviate
liver disease and/or diabetes.Lung transplantation is considered when
lung function declines to the point where assistance from mechanical
devices is required or patient survival is threatened. This point typically
occurs when lung function declines to approximately 20 to 30 percent,
however there is a small time frame when transplantation is feasible as
the patient must be healthy enough to endure the procedure.

Lung Volume Reduction

As noted earlier, the lungs of an emphysematous patient become
hyperinflated with air spaces that contribute little to gas exchange and
the resulting widened chest is difficult for the patient to expand further
when attempting to inhale. By removing lung tissue around dead air
space, surgery can sometimes reduce the patient's work of breathing.
In lung volume reduction surgery, 20% to 30% of the lung volume is
removed from both sides of the chest. As a result, survivors can usually
exercise more than they could before the surgery. Those patients who
have mainly upper-lung emphysema also have an increased lifespan after
this surgery. For other COPD patients, however, longevity is not increased
and it may even be shortened.
The major postoperative complication of lung volume reduction surgery is
significant continuing air leakage from the lungs into the chest. Operative
mortality rates are from 4% to 10% in hospitals providing the procedure.

Bullectomy
Selective removal of individual large empty air spaces (bullae) can
sometimes be carried out using a thoracoscope. In patients with
emphysema, bullae are usually a few centimeters in diameter.
Occasionally, however, bullae can be huge, taking up as much as a third
of the chest space. These giant bullae squeeze the healthier lung tissue
and compress the adjacent blood vessels. By removing giant bullae, the
remaining lung tissue can re-expand and some of the circulation will be
restored. As with lung volume reduction surgery, a major postsurgical
complication of bullectomy is persistent air leakage

Gene Therapy
Gene therapy and has been explored as a potential cure for
cystic fibrosis. Ideally, gene therapy attempts to place a normal copy of
the CFTR gene into affected cells. Transferring the normal CFTR gene into
the affected epithelium cells would result in the production of functional
CFTR in all target cells, without adverse reactions or an inflammation
response. Studies have shown that to prevent the lung manifestations of
cystic fibrosis, only 5–10% the normal amount of CFTR gene expression is
needed. Multiple approaches have been tested for gene transfer, such as
liposomes and viral vectors in animal models and clinical trials. However,
both methods were found to be relatively inefficient treatment options.
The main reason is that very few cells take up the vector and express the
gene, the treatment has little effect. Additionally, problems have been
noted in cDNA recombination, such that the gene introduced by the
treatment is rendered unusable.

Preventive Measures
Cystic fibrosis is a genetic disorder that cannot be prevented.
But people who have cystic fibrosis can help prevent more serious health
problems such as lung infections by:

• Keeping immunizations current. Children who have cystic

fibrosis should have all the recommended immunizations in
addition to pneumococcal and flu shots. For more information on
the recommended schedule of immunizations for children, see the
topic Immunizations.
• Not smoking and avoiding secondhand smoke.
• Using airway clearance techniques, such as postural drainage
and chest percussion.
• Eating nutritious, high-calorie foods.
• Having regular checkups and frequent tests, and following
their treatment plan.
• Seeking care from a cystic fibrosis specialty treatment center,
 if possible.

In addition, parents of a newborn who has been diagnosed with cystic

fibrosis should care for the baby at home and avoid placing him or her in
day care for at least 6 months to 1 year, if possible.

LABORATORY FINDINGS
The key chemistry values for a person with COPD are the levels of
blood gases—oxygen and carbon dioxide—and the pH of the blood.

BLOOD OXYGEN LEVELS

The severity of a patient's COPD can be estimated by the degree
that the blood gases deviate from normal. In the early stages of the
disease the amount of oxygen in arterial blood is usually within normal
limits. Oxygen concentration in arterial blood is measured as its partial
pressure (PaO2), and a normal oxygen partial pressure (or oxygen tension)
is 80 to 100 mm Hg. As COPD worsens, the PaO2 can drop below 60 mm
Hg; this level signals respiratory distress to the brain, and it strongly
activates the respiratory centers. When the PaO2 is below 60 mm Hg, a
person hyperventilates in an attempt to reverse the hypoxemia by
breathing in more oxygen. Unfortunately, hyperventilation due to
hypoxemia expels too much carbon dioxide from the bloodstream, and
this causes respiratory alkalosis, a pH imbalance.
In later stages of COPD, even with a PaO2 <60 mm Hg, the patient
does not have the energy to hyperventilate and carbon dioxide builds up
in the blood. Now the hypoxemia is accompanied by hypercapnia (excess
blood carbon dioxide) and the patient develops chronic respiratory
acidosis, an ominous sign (Sharma, 2006; Swadron & Mandavia, 2006).

Arterial Blood Gases

Early in the course of COPD, arterial blood gases do not need to be
checked regularly. However, an early set of baselines values can be a
used as a comparison to measure the degree of change brought on during
acute exacerbations, such as when the patient gets a serious respiratory
infection.

Pulse Oximetry
Accurately measuring a person's blood oxygen tension requires
drawing arterial blood and testing it in a laboratory. Pulse oximetry is a
quick and noninvasive alternative tool. A pulse oximeter has a small probe
that can be clipped onto a patient's finger or earlobe. Using
measurements of transmitted light, the oximeter determines the percent
of the patient's hemoglobin (Hb) that is saturated with oxygen.
Pulse oximeters are not as accurate as direct oxygen tension
measurements from arterial blood gases, and the percent of hemoglobin
saturation measured by an oximeter is not the same as a person's PaO2.
Nonetheless, the two values are related. A person with a normal PaO2 (80–
100 mm Hg) will have an Hb saturation of ≥96%; a person with
hypoxemia of 60 mm Hg will have an Hb saturation of about 86%.

RED BLOOD CELLS

Routine blood analyses are not needed to manage most cases of
COPD. Some people with severe COPD produce excess red blood cells
(polycythemia), apparently in response to their chronic hypoxia. This
leads to hematocrit readings of >52% in men (normal is 43–52%) and
>48% in women (normal is 37–48%).

ALPHA1-ANTITRYPSIN LEVELS
Patients who develop emphysema at an early age (younger than 40
years old) and nonsmokers of any age who develop emphysema are
usually tested for their blood levels of the enzyme alpha1-antitrypsin
(AAT). Deficiency of this enzyme makes a person unusually susceptible to
emphysematous COPD, and the patient and family should be educated
about this genetic disease. It is sometimes possible to treat AAT-
deficiency with replacement doses of the enzyme.

IMAGING STUDIES
The most commonly used images for evaluating and managing
COPD are chest x-rays and computed tomography (CT) scans.

CHEST X-RAYS
COPD is a disease defined by functional problems, specifically, the
restriction or obstruction of airflow in the lungs. Breathing measurements
are better diagnostic indicators of the disease than are chest x-rays.
Chest x-rays are important, however, for ruling out other causes of airway
obstruction, such as mechanical obstruction, tumors, infections, effusions,
 or interstitial lung diseases. In acute exacerbations of COPD, chest x-rays
are used to look for pneumothorax, pneumonia, and atelectasis (collapse
of part of a lung) (Wise, 2007).
7. Understand, At the severe stage, COPD produces a number of changes visible in
learn and chest x-rays. When the disease includes significant emphysema, the chest
practice the is widened, the diaphragm flattened, and the lung fields have fainter and
Nursing fewer vascular markings. The heart may appear long, narrow, and vertical
Management of and the airspace behind the heart can be enlarged.
the disease. When severe COPD includes significant chronic bronchitis, chest x-
rays have a "dirty" look. There are more vascular markings, more
nonspecific bronchial markings, and the walls of the bronchi look thicker
than normal when viewed end-on. Often, the heart appears enlarged
(Swadron & Mandavia, 2006).

COMPUTED TOMOGRAPHY (CT) SCANS

Computed tomography scans, especially high-resolution scans, are
better than chest x-rays at resolving the details of lung abnormalities
caused by COPD. These scans are also used to more definitive
identification of diseases such as tumors or infections that may be
complicating a patient's COPD. Late in the disease, CT scans are used to
evaluate COPD patients who are to be treated surgically.

LUNG FUNCTION TESTS

Pulmonary function tests can quantify the severity of airway
obstruction in COPD. When COPD is diagnosed, baseline pulmonary
function values should be recorded. Later tests can then be used
objectively to follow the progression of the disease and the effectiveness
of treatments (Gold, 2005a). For COPD, the two general classes of
breathing tests are (1) measurements of effective lung volumes, and (2)
measurements of airflow rates and airflow volumes.

LUNG VOLUMES
In COPD, airway obstruction makes it difficult to empty the lungs
fully. The residual air merely takes up space. The air that remains behind
keeps the lungs hyperinflated even after a complete exhalation, making it
more difficult for a patient to pull in sufficient air for the next full breath.
As a result, the total air volume of the lungs often increases while the
volume of air actually breathed in and out decreases.
The effective volume of air is called the vital capacity (VC), which
is the largest volume of air that can be exhaled after a full inhalation. The
vital capacity is measured by having a patient take as large a breath as
possible and then exhale as quickly and forcefully as possible. The volume
of exhaled air is the vital capacity. When measuring vital capacity this
way, the result is also called the forced vital capacity (FVC) (Wanger &
West, 2005).

AIRFLOW RATES
The airway obstruction of COPD slows the movement of air in the
lungs. This slowing can be measured directly. Measurements of the rate of
air movement during breathing are called spirometric measurements.
Specifically, spirometry records the volume of air exhaled in a defined
period of time (Miller et al., 2005).
A full-function laboratory spirometry apparatus gives detailed reports on a
range of characteristics of a patient's lung ventilation (NIH, n.d.c).
The most common spirometric measurement used to characterize a
patient's COPD is the one-second forced expiratory volume (FEV1).
This is the maximum amount of air that a patient can breathe out in the
first second of a forced exhalation after having taken a full breath.

EVALUATING AIRWAY OBSTRUCTION

People with normal lungs can expel most of the air in their lungs
within 1 to 2 seconds. The amount of air forcefully exhaled in the first
second (the FEV1) is about 3/4 of the vital capacity (the FVC) of a normal
person.
If someone could exhale their entire vital capacity in 1 second, their
FEV1/FVC would be 1.00. A normal person has an FEV1/FVC between 0.70
and 0.80; in other words, a person with normal lungs can exhale between
70% and 80% of their vital capacity in the first second. This ratio,
FEV1/FVC (the percent of the vital capacity that can be exhaled in one
second), declines as a person ages, but even elderly people will have
FEV1/FVC >0.70 if their lungs are normal.
In COPD, airway obstruction restricts the rate of exhaling and people
with COPD cannot get a normal amount of air out of their lungs in one
second. People with COPD have FEV1/FVC <0.70. When a person has an
FEV1/FVC <0.70 and a history of >20 pack-years of smoking, they can be
given a presumptive diagnosis of COPD (Wagner & West, 2005).

RANKING THE SEVERITY OF COPD

The basic stages of COPD are termed mild, moderate, severe, and
very severe. All stages of COPD have an abnormally low one-second
exhaled percent of vital capacity (FEV1/FVC <0.70). The specific stage of
COPD is then determined by how much the disease has reduced the
maximal airflow below normal; this is measured by comparing the
patient's FEV1 to the predicted value for their age, gender, height, and
weight (Swadron & Mandavia, 2006; Wise, 2007)

STAGING OF COPD
Stage Severity FEV1/FVC

Stage I Mild FEV1/FVC <0.70 and FEV1 ≥80% predicted

value*

Stage II Moderate FEV1/FVC <0.70 and 50% ≤FEV1 <80%

predicted value*

Stage Severe FEV1/FVC <0.70 and 30% ≤FEV1 <50%

III predicted value*

Stage Very Severe FEV1/FVC <0.70 and FEV1 <30% predicted

IV

*P re di c t e d F EV 1 v al ue s ad j u st e d f or a p e r so n' s a ge , g e n de r, h e i g ht , an d
w e i g ht c a n be c al c u l a te d fr om p ub l i sh e d e qu at i o ns (Pe l l e g ri no e t a l . ,
2 0 0 5 ).
So ur c e : Mo di fi e d fr om Ra be e t al . , 2 0 0 7 .

NURSING MANAGEMENT
 PHYSICAL EXAMINATION
A patient with mild COPD may have few signs of the disease,
especially when sitting quietly. In contrast, the physical exam of a person
with severe COPD can be diagnostic (Shapiro et al., 2005; Swadron &
Mandavia, 2006).

GENERAL APPEARANCE

Most COPD patients especially with significant emphysema are

typically thin with a barrel-shaped chest. They tend to breathe through
pursed lips, and they sit leaning forward in a "tripod position"; this is a
posture that widens the chest as much as possible by supporting the
upper body on the elbows or the extended arms.

The tripod position. Patient leans forward, resting on elbows

orhands, in an effort to expand the chest and ease breathing.
(Illustration by Jason M. McAlexander, MFA. Copyright © 2007
Wild Iris Medical Education.)

In contrast, patients with significant chronic bronchitis

are typically of normal weight or overweight. They have a
productive cough and may be cyanotic. At rest, their rate of
respirations is high, often more than 20 breaths per minute.
Patients with the chronic bronchitis form of COPD who are hypoxemic may
have a clouded consciousness, making them dull and irritable.

WEIGHT
Obesity can worsen the symptoms of COPD. On the other hand,
many COPD patients—especially patients with the emphysematous form
of COPD—are cachectic and underweight, with muscle wasting. In these
cases, nutritional therapy is an important part of their treatment.

CHEST

A COPD patient with chronic bronchitis but little emphysema may have a
normal-sized chest. Significant emphysema, on the other hand, leads to a
wide, barrel-shaped chest with a flattened diaphragm. In a patient with
emphysema, the chest remains perpetually in the position of inhalation.
To take a new breath, therefore, emphysematous patients must expand
their chests beyond the normal position of inhalation, and this requires
using accessory respiratory muscles of the shoulder, neck, and back.

LUNGS
The chest of an emphysematous patient is unusually resonant to
percussion and the breath sounds are distant. At the other end of the
spectrum, the chest of a chronic bronchitis patient can have dull spots
when percussed and it will be noisy with rales, rhonchi, and wheezing.
The common feature of all forms of COPD is airway obstruction,
which worsens as the disease becomes more severe. A simple, direct
measure of airway obstruction is the time it takes a patient to blow out an
entire lungful of air. A normal person has a forced expiratory time (FET) of
<3 seconds. An FET of >4 seconds suggests obstruction. An FET of >6
seconds indicates considerable airway obstruction, at the level of
moderate-to-severe COPD.

HEART
COPD can injure the heart in two major ways. First, the chronic
inflammatory state of COPD predisposes a person to develop coronary
artery disease; therefore, a heart history and physical examination should
probe for evidence of ischemic heart problems. Second, COPD can cause
pulmonary hypertension, which strains the right ventricle of the heart.
Pulmonary hypertension makes the pulmonary component of the second
heart-sound louder. In addition, it can cause tricuspid valve insufficiency,
which will be heard as a holosystolic murmur loudest along the left sternal
border. Pulmonary hypertension can cause right-sided heart failure (cor
pulmonale), and this will lead to jugular venous distension and edema of
the legs and ankles.

LIFESTYLE ADDITIONS AND MODIFICATIONS

Medications are the fundamental day-to-day tools for controlling the
symptoms of COPD, but there are also four key nonpharmaceutical steps
in the treatment of COPD (Shapiro et al., 2005; Stulbarg & Adams, 2005).

PATIENT EDUCATION
Teach your patients about COPD. Explain that the disease causes
irreversible and progressive problems. Warn patients that they will have
episodes in which the symptoms—difficulty breathing, wheezing,
productive cough and tiredness—get worse for days or even weeks.
Assure patients that you will help them by ordering medications
that make breathing easier. Tell them there are a number of things they
themselves can do to slow the progression of the disease and to lessen
the number of acute exacerbations. The most important of these things is
to stop smoking; although smoking has already damaged their lungs,
continued smoking will increase the damage and will make their COPD
worsen more quickly.
Explain to patients the importance of staying active. In addition, give
them practical suggestions that will help them to cope with the inevitable
limitations posed by COPD. For example, tell them:
• Slow the speed at which you do things, and stop and rest when you
are tired. Don't push yourself.
• Pace yourself and plan your activities for times when you have the
most energy. You will feel best soon after you take your
bronchodilator medicines. Wait an hour after meals before you do
activities.
• Sit on a chair or stool in the shower—don't stand. Likewise, sit while
you shave, comb your hair, and brush your teeth.
• Don't use products that are hard on the lungs; for example, hair
sprays, spray-on deodorants, or strong perfumes.
• Use the exhaust fan in your kitchen to make it less likely that you
will breathe smoke and cooking vapors.
• Wear slip-on shoes so you don't have to bend over to tie laces.
• Make sure your occupation does not require more physical exercise
than you can actually do. Consider setting smaller goals at work and
allowing more time to finish tasks.
• Find out how to get a daily air pollution report, and don't go out on
days with moderate or severe pollution.
• Ask people not to smoke in your home or work area. (ALA, 2007)

STOP SMOKING
In the United States, smoking starts in the teenage years: 90% of
adult smokers began smoking before the age of 18. More than a quarter
of high school students and 1 in 10 middle school students smoke
(Ranney et al., 2006).
Most patients with COPD have a long smoking history and many will still
be smoking when they are under medical care. Currently, the only way to
change the course of COPD is for the patient to stop smoking. No matter
how old they are and no matter how long they have been smoking, COPD
patients benefit from quitting.
COPD is an insidious disease, and it develops over many years before it
compromises people enough that they go to a doctor. The disease is
already active and destructive by the time that it is diagnosed, and
treatment should be aggressive from the beginning. From day one,
strongly urge your patients to stop smoking.
Nicotine is powerfully addictive. In addition, the smoking ritual fills many
basic psychological needs. When doctors merely tell patients to stop
smoking, their patients succeed over the long-term only 5% of the time.
Although simply advising smokers to quit is rarely effective, healthcare
professionals are less likely to offer smoking cessation assistance with
their advice (CDC, 2007).
Long-term success rates of greater than 20% to 40% can be achieved by
comprehensive programs that include behavioral therapy and
medications. Successful smoking intervention programs begin by setting a
quitting date with the patient. They then maintain continued contact with
the patient to provide medication, counseling, support, advice, and a
modicum of social pressure. For specific recommendations, the report
Treating Tobacco Use and Dependence: Clinical Practice Guidelines can
be downloaded from the United States Surgeon General's website at
http://www.surgeongeneral.gov/tobacco.
The pharmacologic aspect of smoking cessation programs attempts to
ease the effects of nicotine withdrawal. Smokers who need their first
cigarette within a half-hour of getting up in the morning are likely to be
highly addicted to nicotine. When these people stop smoking they
become anxious, irritable, easily angered, easily tired, and depressed.
Their sleep is disrupted and they have difficulty concentrating. Withdrawal
effects happen during the first 2 to 3 weeks after quitting.
To lessen withdrawal symptoms, nicotine can be taken directly. Nicotine
replacements are available as gum, lozenges, transdermal patches,
inhalers, and nasal sprays. These should be used on a regular schedule
and also prn (as needed for cigarette cravings) for about two weeks, and
then the doses reduced gradually.
Antidepressants (notably, bupropion and nortriptyline) have been shown
to help patients for whom nicotine replacement therapy has not worked.
In 2006 a nicotine agonist, varenicline (Chantix), was approved by the
FDA for anti-smoking therapy. Varenicline binds to nicotine receptors and
prevents nicotine from activating the receptors while producing a smaller
stimulant effect than nicotine.

PULMONARY REHABILITATION
Pulmonary rehabilitation is the term for a group of techniques used
to improve patients' conditioning and ease their breathing difficulties.
Pulmonary rehabilitation is done as outpatient therapy. Some programs
continue for an extended time, but most run for a few weeks and then
give patients individualized instructions for continuing at home. Education
sessions are important parts of rehabilitation programs; in these sessions,
patients and their families learn details about COPD and its treatment
(Chesnutt et al., 2008).
Pulmonary rehabilitation programs are tailored to the needs of each
individual. Typically, they include graded aerobic exercise programs such
as regular sessions of walking or stationary bicycling three times weekly.
A walking exercise program, for example, might begin with slow treadmill
walking for only a few minutes. The length and speed of the walking
would then be increased gradually over 4 to 6 weeks. The goal would be
for the patient to walk for 20 to 30 minutes without needing to stop
because of shortness of breath. At that point, the patient would be
assigned a maintenance exercise program to be done at home.
Rehabilitation sessions also include exercise routines to condition the
upper body and exercises aimed at strengthening respiratory muscles.
Breathing instruction teaches patients how to slow their rate of breathing
by pursing their lips. Patients also learn how to give their upper
respiratory muscles a rest by using abdominal breathing techniques
instead of chest breathing.
Comprehensive pulmonary rehabilitation can reduce COPD patients'
symptoms and increase the amount of exercise that patients can do
without being stopped by dyspnea. It can also reduce the number of
hospitalizations for acute exacerbations, and improve the quality of
patients' lives, both subjectively and objectively (Stulbarg & Adams,
2005).

PHYSIOTHERAPY
 It is important that COPD patients with significant chronic bronchitis
keep their airways clear. Coughing up sputum should be encouraged, and
cough suppressants or sedatives should not be used routinely. When
patients cannot clear their secretions by coughing, postural drainage may
help (Stulbarg & Adams, 2005).
Most people's lungs secrete extra mucus in response to inhaled irritants.
To avoid stimulating excess secretion, COPD patients need to avoid
smoke-filled rooms and stay indoors during air pollution alerts. Home air
conditioners and air filters are effective and helpful.

IMPROVED DIET
Overweight COPD patients find improvement when they slim down.
Some COPD patients, however, have the opposite problem: they become
thin and malnourished. In part, cachexia results from the high-energy cost
of breathing with COPD. In addition, the chronic inflammatory state
underlying COPD tends to put the body's metabolism into a catabolic
state. To help them maintain a healthy body weight, thin COPD patients
should be given dietary counseling that includes specific
recommendations of meals that are nutritionally balanced and that
contain sufficient calories to make up for the work of breathing (Stulbarg
& Adams, 2005; Wise, 2007).
.
BIBLIOGRAPHY:

BOOKS:

Rod R. Seeley, Trent D. Stephens, and Philip Tate, ESSENTIALS OF ANATOMY AND PHYSIOLOGY.
Copyright 2007 Edition. Mc-Graw Hill Education (Asia).

WEB SOURCES:

http://nursingcrib.com/nursing-notes-reviewer/cystic-fibrosis/
http://health.yahoo.com/respiratory-prevention/cystic-fibrosis-prevention/healthwise--hw188763.html
http://en.wikipedia.org/wiki/Cystic_fibrosis

http://ask.reference.com/web?qsrc=2417&o=10616&l=dir&q=pseudomonas+aeruginosa

http://www.medscape.com/medline/abstract/9759549?prt=true

http://en.wikipedia.org/wiki/Pseudomonas_aeruginosa

http://www.nursingceu.com/courses/217/index_nceu.html
APPENDIX 1

PATHOPHYSIOLOGY
RISK FACTORS
• HEREDITY
• LIFESTYLE
• ENVIRONMENT

EFFECTS ON THE
RESPIRATORY
SYSTEM:
MUCUSAL BUILD – UP

TRIGGERS INFLAMMATORY
PROCESS
BRONCHOCONSTRICTION AND CLOGGING OF
AIRWAYS

BRONCHIAL TRIGGERS COUGH INCREASED MUCUS ATTRACTS BACTERIA CAUSES INFECTION

IRRITATION REFLEX PRODUCTION / IN THE PLEURAL
COPIOUS PHLEGM CAVITY
PRODUCTION (PNEUMONIA)

INCREASE PLEURAL PRESSURE HEMOPTYSIS and

BRONCHOECTASTASIS
PULMONARY HYPERTENSION
BLOCKAGE OF RESPIRATORY
CHRONIC HEART FAILURE SINUSES FAILURE
 PAIN, FEVER, NASAL
DRAINAGE (SIGNS
OF LOCAL
INFECTION)

GROWTH OF NASAL POLYPS

BLOCKS NASAL
PASSAGES

INCREASES BREATHING
DIFFICULTIES

CHRONIC OBSTRUCTIVE PULMONARY

DISEASE: CYSTIC FIBROSIS