CLASS ROOM TEACHING

CYSTIC FIBROSIS
INTRODUCTION :
Cystic fibrosis is the most common fatal autosomal recessive disease among
the Caucasian population .a person must inherit a defect copy of the CF gene
(one from each parent ) to have CF .although CF was once considered a fatal
childhood disease , approximately 37% of people living with the disease are 18
years of age or older. Researchers have documented survival past age 76 years
This multisystem genetic disease is usually diagnosed in
infancy or early childhood but may be diagnosed later in life . respiratory
symptoms are frequently the major manifestation of CF when it is diagnosed
later in life .
ETIOLOGY :
Genetic predisposition and disorder in gene mutation .

ANATOMY & PHYSIOLOGY :Upper respiratory tract:

Nose The nose is composed of an external & an internal portion. The
external portion protrudes from the face & is supported by the nasal bones &
cartilage. The nose serves as a passageway for air to pass to & from the
lungs. It filters impurities & humidifies & warms the air as it is inhaled.

Para nasal sinuses- The Para nasal sinuses include four pairs of bony
cavities that are lined with nasal mucosa & ciliated pseudo stratified
columnar epithelium.

Turbinate bones The turbinate bones, or conches, are adapted by shape &
position to increase the mucous membrane surface of the nasal passages & to
obstruct the air flowing through them slightly.

Pharynx, tonsils, & adenoids The pharynx or throat is a tube like

structure that connect the nasal & oral cavities to the larynx..

Larynx The larynx or voice organ is a cartilaginous epithelium lined

structure.
Lower respiratory tract:

Lungs The
lungs
are
paired elastic
structures
enclose
in
the thoracic
cage, which
is an airtight
chamber
with
distensible
walls
ventilation requires movement of the walls of the thoracic cage & of its
floor, the diaphragm.
There are two lungs one lying on each side of the midline of the thoracic cavity
they are cone shaped & have an apex, base, costal surface & midline surface.
Apex :This is rounded & rises into the root of the neck about 25 mm above the level of
the middle third of clavicle. It lies close to the first rib & the blood vessels &
nerve in the root of the neck
Base :This is concave & semi lunar in shape & rice on the thoracic surface on the
diaphragm.
Costal surface :The surface is convex & lies against the costal cartilages, the ribs & the
intercostals muscles.

Medial surface :This surface is concave & has a roughly triangular shaped area, called hilum at
the level of the 5th, 6th & 7th thoracic vertebrae.
Associated structure of lungs :The area between the lungs in the mediastinum. It is also occupied by the heart,
great vessels, trachea, right & left bronchi esophagus, lymph nodes, lymph
vessels & nerve.

The right lungs is divided into three distinct lobes superior, meddle & inferior.
The left lung is smaller because the heart occupies space left of the midline. If is
divided into two lobes superior & inferior.
Functions of the respiratory system the function of respiratory system are
given below:
Oxygen transport oxygen is supplied to, & carbon dioxide is
removed from, cells by way of the circulation blood. Cells are in close
contact with capillaries, whose thin walls permit easy passage or
exchange of oxygen & carbon dioxide.
Respiration after these tissue capillary exchanges, blood enters the
systemic veins & travels to the pulmonary circulation.
Ventilation during inspiration, air flows from the environment into
the trachea, bronchioles, & alveoli.
PATHOPHYSIOLOGY :

Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane

conductance regulator protein , which is a chloride channel found in all
exocrine tissues.

Chloride transport problems lead to thick , viscous secretions in the lungs ,

pancreas , liver, intestine ,and reproductive tract as well as increased salt
content in sweat gland secretions .

CLINICAL MANIFESTATIONS :
The pulmonary manifestations of CF includes ;
1.
2.
3.
4.

Productive cough
Wheezing
Hyperinflation of the lung fields on chest x- ray ,
Chronic respiratory inflammation and infection are caused by impaired
mucus clearance,
5. Colonization of the airways with pathogenic bacteria usually occurs early
in life ,Staphylococcus Aureus and H. Influenza are common organisms
during early childhood ,as the disease progresses , Pseudomonas
Aeruginosa is ultimately isolated from the sputum of most patients .
6. Upper respiratory manifestations of the disease include sinusitis and
nasal polyps ,
Non-pulmonary clinical manifestations include ;
Gastrointestinal problems are ;

Pancreatic insufficiency
Recurrent abdominal pain
Biliary cirrhosis
Vitamin deficiencies
Recurrent pancreatitis
Weight loss

Other ; CF related diabetes ,

Genitourinary problems like male and female infertility,
Clubbing of the digits ( fingers $ toes ).
ASSESSMENT AND DIAGNOSTIC FINDING :
The diagnosis of is suspected in patients with typical clinical features of CF
;once other diseases have been excluded .
Elevated sweat chloride concentration , repeated sweat chloride values of
greater than 60meq/l distinguish most people with CF from those with other
obstructive diseases .
The only acceptable procedure for sweat testing is the quantitative Pilocarpine
Iontophoresis sweat test .it should be performed in a laboratory that
frequently does this test .

MANAGEMENT :
Pulmonary problems remain the leading cause of morbidity and mortality in CF
and account for death in more than 95%of patients , a variety of management
techniques are necessary .
Because chronic bacterial infection of the airways occurs in CF , control of
infections is essential to treatment . antibiotic medications are routinely
prescribed for acute pulmonary exacerbations of the disease . depending on
the severity of the exacerbation , aerosolized , oral , or iv antibiotic therapy
may be used .
Antibiotic agents are selected based on the results of a sputum culture and
sensitivity .
Bronchodilators , including beta2 adrenergic agonists and anticholinergics,
are frequently administered to treat airway hyperactivity and to reverse
bronchospasm .
Airway clearance is the key intervention in the care of patients with CF , and
various pulmonary techniques are used to enhance secretion clearance ,
example include;

manual postural drainage

chest physical therapy ,
high frequency chest wall oscillation
autogenic drainage ( a combination of breathing techniques at different
lung volume levels to moves the secretions to where they can be huffcoughed out.
Other devices that assist in airway clearance ,such as masks that
generate positive expiratory pressure (PEP masks)
Inhaled mucolytic agents such as N-acetylecysteine (mucomyst)may also
be used .
These agents help decrease the viscosity of the sputum and promote
expectoration of secretion .
To decrease the inflammation and on going destruction of the airways ,anti
inflammatory agents ,including inhaled corticosteroid or systemic therapy ,may
also be used .
Corticosteroids are used in late stage disease and during severe respiratory
exacerbations .

Supplemental oxygen is used to treat the progressive hypoxemia that occurs

with CF .
Lung transplantation is an option for a small , selected population of patients
with CF . A double lung transplantation technique is used because of chronic
infection of both lungs in end stage CF .
Recent study : gene therapy is a promising approach to management , and
clinical trials are underway . it is hoped that techniques will be developed to
carry healthy genes to the damaged cells and correct defective CF cells. efforts
are underway to developed to carry healthy genes to the damaged cells and
correct defective CF cells.
NURSING MANAGEMENT :
Nursing is crucial to the interdisciplinary approach required for care of adults
with CF .
1)Assessment :
Assess for breathing rate , rhythm , chest expansion $ pulmonary congestion
,auscultate for any moist crackles , rhonchi, check for neck vein congestion,
pulmonary artery pressure , measure CVP.
Nursing diagnosis :
Impaired gas exchange r/t pulmonary congestion and bronco-constriction and
excessive secretion .
EOC:
Improvement of gas exchange .
Interventions:
Assess for breathing pattern by means of
checking ;

1.

Respiratory rate
Rhythm
Chest expansion
dyspnoea
moist crackles on auscultation
Provide comfortable position e.g propped up position

2. Administer oxygen initially but as the distress progress intubate, and start
mechanical ventilation ,monitor closely for secretions .
3. Administer Bronchodilators as prescribed
4. Nebulise with the order of doctor .
5. Chest physiotherapy including postural drainage , chest percussion
vibration , and breathing exercises.
Evaluation :
May improve from dyspnoea with support of ventilator and ABG analysis show
improvement in oxygenation .
2)Assessment : Assess for activity tolerance power of the patient .
Nursing Diagnosis :
Activity intolerance due to fatigue , hypoxemia , ineffective breathing pattern
and excessive secretion .
EOC;
Improvement in activity tolerance .
Nursing interventions:
1)Encourage the patient to move about when in bed and teach passive
exercises .
2)Change the position every 2 hourly to promote blood circulation .
3)support the patient in establishing a regular regime of care which includes all
the necessary care as well as appropriate exercise .
4)If the patient is disturbed than diversional activity will be beneficial.
5)Consult with a occupational therapist if prolonged immobility and inactivity
are anticipated .
6)If the activity is disturbed by chest pain than administer analgesic before any
scheduled activity because the pain may be due to intubation and mechanical
ventilation .
Evaluation :

May progress gradually to optimal level of activity with all support from health
personnel.
3) Assessment :
Assess the patients coping ability by means of;
establishing good
interpersonal relationship , patients interest for treatment as well as
expression of the patient towards the life .
Nursing diagnosis :
Ineffective coping related to reduced socialization , anxiety , depression ,
lower activity level and inability to work .
EOC :
Attainment of an optimal level of coping .
Nursing Interventions :
1. Help the patient to accept realistic goals that will promote a sense of
hope and accomplishment rather than defeat and hopelessness.
2. Encourage activity to level of symptom tolerance which reduces tension
and decreases degree of dyspnoea as patient becomes conditioned .
3. Teach relaxation technique or provide a relaxation tape for patient
which reduces tension, stress, anxiety and dyspnoea and helps patient
to cope with disability .
4. If available encourage the patient to participate in pulmonary
rehabilitation centre which shown to promote subjective improvement
in a patients status and self esteem as well as increase exercise
tolerance and decreased hospitalization .
Evaluation :
Patient may develop an optimal level of coping mechanism and may
demonstrate faster recovery .
4) Assessment :
Assess the patients knowledge regarding the home care and compliance to
treatment by asking questions and listening to the patients doubts .

Nursing Diagnosis :
Deficient knowledge about self management to be performed at home .
EOC : Adherence to therapeutic program and home care .
Nursing interventions :
1) Help patient identify / develop short and long term goals
Teach the patient about disease , medications , procedures , and how
and when to seek help
Refer the patient to pulmonary rehabilitation
2)Teach postural drainage if appropriate
3) Teach patient to co-ordinate diaphragmatic breathing with activity .
4)Advice the patient as well as relatives to adhere with the treatment
regime and seek advice from the doctor as needed .
5)Advice the patient to carry out daily activities as per the activity tolerance
power and take adequate rest .
Evaluation :
The patients knowledge may increase after all explanation .
PATIENT EDUCATION :
Patient education is a major component of pulmonary rehabilitation and
includes a broad variety of topics . The topics may include normal anatomy
and physiology of the lung and changes with cystic fibrosis since it is a
genetic problem .
The other topics includes home oxygen therapy, medication ,nutrition ,
respiratory therapy treatments , symptom alleviation and coping with
chronic disease .
Breathing exercises :
The breathing pattern of most people with cystic fibrosis is shallow , rapid
and inefficient so this type of upper chest breathing can be changed to

diaphragmatic breathing , which reduces the respiratory rate , increases

alveolar ventilation .
Pursed lip breathing helps slow expiration , prevents collapse of small
airways and help the patient control the rate and depth of respiration .it
also promotes relaxation , enabling the patient to gain control of dyspnea
and reduce feeling of panic .
Inspiratory muscle training :
Once the patient master the diaphragmatic breathing , a program of
Inspiratory muscle training may be prescribed to help strengthen the
muscle used in breathing . This program involves use of respiratory training
device , which is placed in the mouth . The patient is instructed to inhale
against a set resistance for a prescribed amount of time every day .
As the resistance is gradually increased , the muscle become better
conditioned .
Activity pacing: Since the patient will have decreased exercise tolerance so
help the patient for planning self care activities and determining the best
times for bathing , dressing , and other daily activities .
Oxygen therapy :
Help the patient to adhere to the oxygen prescription , explain the patient
proper flow rate and required number of hours for oxygen use at home .
Nutritional therapy :
Nutritional assessment and counselling are important aspects in the
rehabilitation process for patient with cystic fibrosis .weight loss as well as
depletion of muscle mass may be observed in stable patients with cystic
fibrosis , so there is need for continuous monitoring if weight recording as
well as a well balanced diet which is rich in calorie because the patient
needs more energy for daily activities to carry out .
CONCLUSION : So Cystic fibrosis is the most common fatal autosomal
recessive disease among the Caucasian population .a person must inherit a
defect copy of the CF gene (one from each parent ) to have CF .

BIBLIOGRAPHY

Black m. Joyce $ Hawks Hokanson Jane medidical surgical nurding ,vol; 2 ,elsevier 2009,
pp 832 -834
Brunner and suddharth medical surgical nursing 11th ed.2009, lippincott,pp 739 -752.
Brunner and suddharth medical surgical nursing 10 th ed.2009, lippincott,pp 739 -752.
Shafers phipps long woods medical surgical nursing 7th ed.1996 pp 1771-7

Http www.health a to z.com

CLASS ROOM TEACHING ON CYSTIC FIBROSIS

Name of student :
Name of the guide :
Topic :

Cystic Fibrosis

Group :
Venue :
Date :
Time :
A.V Aids:
Method of teaching :

LCD , OHP, Poster , Blackboard

Lecture Cum Discussion

Previous knowledge : The students will have some knowledge regarding the
topic .
General objectives : At the end of the class the student will have knowledge
about Cystic Fibrosis and will apply the knowledge in the clinical practice .
Specific objectives : at the end of the class the students will be able to ;
a)
b)
c)
d)
e)
f)
g)
h)

define Cystic Fibrosis

enumerate the etiology of CF
discuss the causes of CF
explain the pathophysiology of CF
list down the clinical manifestations of CF
describe the assessment and diagnostic findings of CF
discuss the management of CF
describe the nursing management of CF

SAIMS College of
Nursing, Indore

SEMINAR ON CYSTIC FIBROSIS

Submitted to :
Dr. Mrs. Meenakshi Krishnan
Principal
SAIMS College of Nursing
Indore

Submitted By :
Miss ..
M.Sc. Nursing
Final Year Student