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

1. Mr. Ravi Rai Dangi
Assistant Professor
Cystic Fibrosis
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2. Outlines
• Introduction
• Risk factors
• Pathophysiology
• Clinical sign and symptoms
• Diagnostic investigation
• Management
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3. 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.
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4. • 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.
Definition
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6. • 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.
• 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.
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Etiology & Risk factors

7. CFTR loss of its function as a chloride ion transporter caused by misfolding
protein
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Pathophysiology
Abnormal sodium and chloride transport across cell membrane,
causing thick tenacious secretion in lung and pancreases
Thick, sticky respiratory secretions that are difficult to remove
cause airway obstruction, resulting in air trapping and frequent
respiratory infection.

8. • Coughing
• Increased mucus in the sinuses or lungs
• Fatigue
• Nasal congestion caused by the nasal polyps
• Recurrent episodes of pneumonia.
• Loss of appetite
• Abdominal distention
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Clinical Manifestation

9. • Failure to pass stool
• Pneumothorax ~10% of CF patients
• Hemoptysis
• Respiratory failure
• Protein and fat malabsorption
• Frequent bulky, foul-smelling stools
• VitaminA, D, E, K malabsorption
Clinical Manifestation
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10. Symptoms in newborns may include :
• Delayed growth
• Failure to gain weight
• No bowel movement (meconium)
• Salty-tasting skin
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Clinical Manifestation

11. • Newborn screening test
• Genetic testing
• Sputum tests
• Computerized tomography (CT).
• Magnetic resonance imaging (MRI)
• Sweat test: sweat chloride values of greater than 60 mEq/L
• Genetic tests to find out what type of CFTR defect is causing CF.
• Chestx ray: Inflated lung, lungs fibrosis and scaring
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Diagnostic Tests

12. • Antibiotics to prevent and treat lung and sinus infections
Eg:Azithromycin
• Anti-inflammatory medications may help reduce the inflammation in
your lungs that is caused by ongoing infections. Eg:Steroid and
Ibuprofen.
• Inhaled medicines to help open the airways. (eg:Mucolytics like
Bisolven®)
• Gene therapy. attempts to place a normal copy of the CFTR gene into
affected cells
• Oxygen therapy may be needed as lung disease gets worse.
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Medical Treatment

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Medical Treatment
Cystic fibrosis has no cure. The goals of CF treatment include:
• Preventing and controlling lung infections
• Loosening and removing thick, sticky mucus from the lungs
• Preventing or treating blockages in the intestines
• Providing enough nutrition
• Preventing dehydration

14. Surgical Treatment
• Lungs transplantation requires surgery to replace one or both of your
lungs with healthy lungs from a human donor.
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15. • Assess respiratory status, including vital signs, breath sounds, Sao2 and
skin color at least every 4 hourly
• Assess cough and sputum (amount, color, consistency and possible
odor).
• Monitor ABG results: report increasing hypoxemia and other abnormal
result to the physician
• Place the patient in the high fowler’s position.
• Encourage frequent position change and ambulation as allowed
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Nursing Management

16. • Assist to cough, deep breathe and use assistive devices.
• Provide endotracheal suctioning using aseptic technique as ordered
• Work with the physician and respiratory therapist to provide
pulmonary hygiene measures, such as postural drainage, percussion
and vibration.
• Administer prescribed medicines as ordered and monitor the effects.
• Spend time with the client and family.
• Answer question honestly
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Nursing Management

17. • Encourage the family to express their feelings, fears and concerns.
• Assist with the understand the grieving process and acceptance of
feelings as normal.
• Help the client and family make decisions regarding treatment and
care.
• Encourage use of other support systems, such as spiritual and social
groups
• Discuss advance directives (the living will) and power of attorney for
health care with the client and family
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Nursing Management

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