cystic fibrosis

1. Care client’s with respiratory disorder
CYSTIC FIBROSIS

2. 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.

3. 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.

4. 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.

5. 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.

7. 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.

8. CLINICAL MANIFESTATION
• Symptoms related to the lungs and sinuses
may include :
 Coughing or increased mucus in the sinuses or lungs
 Fatigue
 Nasal congestion caused by the nasal polyps
 Recurrent episodes of pneumonia.

9. CON’T…
Symptoms in newborns may include :
Delayed growth
Failure to gain weight
No bowel movement (meconium)
Salty-tasting skin

10. INVESTIGATION
• Newborn screening test
• Genetic testing
• Sweat test.
• Sputum tests

11. CON’T…
• Computerized tomography (CT).
• Magnetic resonance imaging (MRI)
• Chest x-rays

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

13. CON’T…
2.Anti-inflammatory medications may help
reduce the inflammation in your lungs that is
caused by ongoing infections.
Eg:Steroid and Ibuprofen.

14. CON’T…
3.Inhaled medicines to help open the
airways. (eg:Mucolytics like Bisolven®)

15. CON’T…
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.

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

17. 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

18. CON’T…
• Digestive complication
• Nutritional deficiencies
• Diabetes
• Blocked bile duct

19. 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.

20. 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.

21. CON’T…
• Encourage patient
drinking plenty of fluids.
• Encourage patient to do exercising two or
three time a week.

23. NURSING CARE PLAN 1
• Nursing diagnosis : Ineffective airway
clearance related to excess tenacious mucus.
• Expected outcome : To improve pulmonary, GI
and pancreatic status.

24. 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 To decrease the lungs work.
and/or diuretics
Encourage coughing, deep breathing exercise Promote lungs expansion, mobilization, and
and frequent position changes drainage of secretions.
Monitor respiratory rate, depth and work of Respiratory may remain somewhat rapid
breathing because hyperthyroid state, but development
of respiratory distress is indicative of tracheal
compression from edema or hemorrhage.
Assist with repositioning, deep breathing Maintains clear airway and ventilation.
exercises, and coughing as indicated

25. NURSING CARE PLAN 2
• Nursing diagnosis : Imbalanced nutrition
related to poor appetite and increased calorie
expenditure.
• Expected outcome : The patient’s weight will
be stable at desired weight for height

26. 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 Eating takes a lot of energy and resting can
rest periods and bronchodilator treatments help conserve energy before meal.
before meals.
Create a pleasant eating environment. Unpleasant views or odors can spoil an
appetite.
Provide smaller and more frequent meals of Eating a lot at one time can fill up the stomach
the patient’s favourite food. and reduce the room for lung.
Encourage family members to bring favourite A large tray of unappetizing food may be more
foods from home for hospitalized patient. than a patient can handle and may spoil the
appetite.

27. 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.

28. Nursing Intervention Rational
Remain with the patient who is acutely Feeling alone during episodes of dyspnea can
dyspneic and anxious. increased anxiety.
Calmly remind the patient to breath slowly in During acute episodes of dyspnea, the patient
through the nose and out through pursed lips. may forget that breathing exercises can help.
Teach deep breathing exercises during times Relaxation can help reduce muscle tension
when anxiety is minimal and remind the patient and distract the patient.
to use them during acute anxiety.
Administer anti anxiety medication. Medication can reduce anxiety but can also
depress respiration, so should be use with
caution.

29. 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.

30. REFERENCE
1. Texts Book:
a. Linda S.WILLIAMS, paula D.HOPPER., Understanding
medical surgical nursing vol 1, 3rd 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/cy
stic_fibrosis.html#
c.http://www.cff.org/treatments/Therapies/
d.http://www.youtube.com/