Care clients with respiratory disorder

CYSTIC FIBROSIS

MUHAMMAD SHOAIB
STMU ISLAMABAD

INTRODUCTION
Cystic fibrosis also known as CF or mucoviscidosis is a
common recessive genetic disease which affects the
entire body,causing progressive disability and often early
death.
Approximately 30,000 Americans
have CF, making it one of the
most common life-shortening
inherited diseases in United States.

DEFINITON
Cystic fibrosis is a disease passed down through families
that cause thick, sticky mucus to build up in the lungs,
digestive tract and other areas of the body.
It is one of the most common
chronic lungs disease in children
and young adults.

ETIOLOGY
Caused by a mutation in the gene for the protein
cystic fibrosis transmembrane conductance
regulator (CFTR).
This mucus builds up in the
breathing passages of
the lungs and in the pancreas,
the organ that helps to break
down and absorb it.

RISK FACTOR
Family history. Because cystic fibrosis is an inherited
disorder, it tends to run in families.
Race. Although cystic fibrosis occurs in all races, it is
most common in white people of northern European
ancestry.

PATHOPHYSIOLOGY
CFTR loss of its function as a chloride ion transporter caused by misfolding protein

Abnormal sodium and chloride transport across cell membrane,causing thick

tenacious secrection in lung and pancrease

Thick,sticky respiratory secretions that are difficult to remove

cause airway obstruction,resulting in air trapping and frequent respiratory

infection.

Cellular Causes of Cystic

Fibrosis
Cystic Fibrosis is caused by a mutation of the gene that contains
the instructions to create cystic fibrosis transmembrane
conductance regulator (CFTR). This mutation causes the sequence
of amino acids in this protein to be assembled wrong, which in
turn causes the entire protein not to fold correctly. When the
incorrectly folded protein enters the Endoplasmic Reticulum (ER),
the ER recognizes the incorrect folding of the protein and marks it
to be degraded. Thus, the protein never even reaches the Golgi,
let alone the cell membrane where it belonged. Since the protein
was intended to be a chloride ion channel through the plasma
membranes of cells that line major passageways in the body
(such as airways and intestines) , the sodium-chloride
concentrations are thrown off throughout the persons body. This
ultimately causes the normally thin mucus within ones body to
turn thick and sticky, and therefore immovable by cilia that line
the passageways.

CONT
Symptoms in newborns may include :
Delayed growth
Failure to gain weight
No bowel movement (meconium)
Salty-tasting skin

Symptoms of Cystic
Fibrosis
Respiratory:
A persistent cough that produces thick mucus
Wheezing or lack of breath
A lowered ability to do exercise
Repetitive lung infections
A persistent stuffy nose and inflamed nasal passages
Digestive:
Foul smelling and greasy stools

Unusually small amount of weight gain or growth

Intestinal blocking, especially in newborns
Severe constipation
Other:
Infertility is common in both males and females, though more frequently in
males

Salty tasting skin and sweat.

Here is a
more visual
representatio
n of many of
the problems
caused by
Cystic
Fibrosis.

Diagnosis
There are many ways for a doctor to diagnose CF. Now all
infants are screened for it in the United States, but as this
was only recently implemented older children or adults
suffering from the previously mentioned symptoms may
be recommended to be screened as well.
Methods of Diagnosis:
Blood from infants can be tested for high levels of the
chemical IRT.
A sweat test can be performed testing a sample of sweat.
Abnormally high levels of salt can indicate that the person
has CF.
Genetic tests can also show CF by doctors observing the
specific malfunctions on the gene actually responsible for the
disorder.

Cellular Causes lead to

Symptoms
Thick mucus build up occurs
due to cell malfunctioning
from out of balance sodium
and chloride concentrations.
The mucus builds up in lungs
and other passageways
because it can not be moved
by cilia. As the mucus builds
up it traps bacteria which
cause chronic lung infections
to develop. The built-up
mucus also blocks airways
and intestines creating
difficulty breathing and
severe constipation.

CONT
Computerized tomography (CT).
Magnetic resonance imaging (MRI)
Chest x-rays

MEDICAL TREATMENT
1.Antibiotics to prevent and treat lung and sinus
infections Eg:Azithromycin

CONT
2.Anti-inflammatory medications may help
reduce the inflammation in your lungs that is
caused by ongoing infections.
Eg:Steroid and Ibuprofen.

CONT

3.Inhaled medicines to help open the

airways. (eg:Mucolytics like Bisolven)

CONT
4.Gene therapy. attempts to place a normal copy
of the CFTR gene into affected cells
5.Oxygen therapy may be needed as lung
disease gets worse.

SURGICAL TREATMENT
Lungs transplantation requires surgery to
replace one or both of your lungs with
healthy lungs from a human donor.

COMPLICATION
Many different types of complication can occur
with CF. The most complications affect the
respiratory,and digestive tract.
Respiratory system include :
Bronchiectasis
Chronic infection
Collapsed lungs
Nasal polyps

CONT

Digestive complication
Nutritional deficiencies
Diabetes
Blocked bile duct

PREVENTION
Actually,there is no way to prevent cystic fibrosis
because it is genetic disorder.
But,if people or their partner has close relatives who
have cystic fibrosis,they both might want to undergo
genetic testing before having children of they own.
If woman already pregnant and the test shows that
her baby may be at risk of cystic fibrosis, doctor
can conduct additional tests on her developing child.

HEALTH TEACHING
Encourage patient avoid smoke, dust, dirt,
fumes, household chemicals, fireplace smoke.

Teach patient clearing or bringing up mucus or

secretions from the airways.

CONT
Encourage patient
drinking plenty of fluids.

Encourage patient to do exercising two or

three time a week.

NURSING CARE PLAN

FOR CLIENT
WITH CYSTIC FIBROSIS

NURSING CARE PLAN 1

Nursing diagnosis : Ineffective airway
clearance related to excess tenacious mucus.
Expected outcome : To improve pulmonary, GI
and pancreatic status.

Nursing Intervention

Rational

Encourage regular exercise

Exercise helps maintain physical

wellness and supplements the
patients airways clearance strategies
by helping to loosen pulmonary
secretion.

Administer oxygen therapy and

receive digoxin and/or diuretics

To decrease the lungs work.

Encourage coughing, deep breathing

exercise and frequent position
changes

Promote lungs expansion,

mobilization, and drainage of
secretions.

Monitor respiratory rate, depth and

work of breathing

Respiratory may remain somewhat

rapid because hyperthyroid state, but
development of respiratory distress is
indicative of tracheal compression
from edema or hemorrhage.

Assist with repositioning, deep

breathing exercises, and coughing as
indicated

Maintains clear airway and

ventilation.

NURSING CARE PLAN 2

Nursing diagnosis : Imbalanced nutrition
related to poor appetite and increased calorie
expenditure.
Expected outcome : The patients weight will be
stable at desired weight for height

Nursing Intervention

Rational

Monitor food intake and weekly

weight.

Regular monitoring can help identify

nutriton problem that are severe.

If the patient is too dyspneic to eat,

schedule rest periods and
bronchodilator treatments before
meals.

Eating takes a lot of energy and

resting can help conserve energy
before meal.

Create a pleasant eating

environment.

Unpleasant views or odors can spoil

an appetite.

Provide smaller and more frequent

meals of the patients favourite food.

Eating a lot at one time can fill up the

stomach and reduce the room for
lung.

Encourage family members to bring

favourite foods from home for
hospitalized patient.

A large tray of unappetizing food may

be more than a patient can handle
and may spoil the appetite.

NURSING CARE PLAN 3

Nursing diagnosis : Anxiety related to dyspnea.
Expected outcome :
The patient will state anxiety is controlled.
The patient will be able to use technique to
control dyspnea and anxiety when they occur.

Nursing Intervention

Rational

Remain with the patient who is acutely Feeling alone during episodes of
dyspneic and anxious.
dyspnea can increased anxiety.

Calmly remind the patient to breath

slowly in through the nose and out
through pursed lips.

During acute episodes of dyspnea,

the patient may forget that breathing
exercises can help.

Teach deep breathing exercises during

times when anxiety is minimal and
remind the patient to use them during
acute anxiety.

Relaxation can help reduce muscle

tension and distract the patient.

Administer anti anxiety medication.

Medication can reduce anxiety but

can also depress respiration, so
should be use with caution.

CONCLUSION
Cystic fibrosis transmembrane conductance
regulator(CFTR) play important role in
function as a chloride ion transporter.
Although there is no cure for CF, treatments
have improved greatly in recent years. Until
the 1980s, most deaths from CF occurred in
children and teenagers. Today, with improved
treatments, some people who have CF are
living into their forties, fifties, or older.

REFERENCE

1. Texts Book:
a. Linda S.WILLIAMS, paula D.HOPPER., Understanding
medical surgical nursing vol 1, 3 rd edition international
edition.
2.The webside about cystic fibrosis:
a.http://www.medicinenet.com/cystic_fibrosis/article.htm
b.http://kidshealth.org/teen/diseases_conditions/digestive/c
ystic_fibrosis.html#
c.http://www.cff.org/treatments/Therapies/
d.http://www.youtube.com/