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Cystic FibrosisEDEN BETH B. ESPINO

BSN3-AUSA-CN

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Cystic Fibrosis

also known as mucoviscidosis

is an autosomal recessive genetic disorder that affects

most critically the lungs, and also the pancreas, liver,

and intestine. It is characterized by abnormal transportof chloride and sodium across an epithelium, leading to

thick, viscous secretions.

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PATHOPHYSIOLOGY

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Predisposing

factors:

heridityage

gender

family history

race

Precipitating

factors:

smokinglung infections

electrolyte

imbalance

Diabetes

pulmonary

hypertension

Mutation in the gene of Cysticfibrosis transmembraneconductance regulator (CFTR)

Body produces thick, stickymucus and it will clogs the lungsthus causing stagnant mucus

Obstructs thepancreas

Malabsorption &

malnutrition

Cystic fibrosis

infected cells creates problems

in the respiratory and digestive

tracts

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Signs and Symptoms

Salty skin

Persistent coughing

Shortness of breath Wheezing

Poor weight gain in spite of excessive appetite

Greasy, bulky stools Nasal polyps: small fleshy growths found in

the nose

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Diagnostic Tests

Sweat Test

Genetic Analysis

Organ function tests Sputum culture

Lung function tests

Imaging tests

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MEDICAL MANAGEMENT

Antibiotics

Mucus-thinning drugs

Bronchodilators

Pain relievers

Oral pancreatic enzymes and better nutrition

Breathing devices

Oxygen therapy

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SURGICAL MANAGEMENT

Lung transplant

Bronchial airway drainage

Nasal polyp removal

Bowel surgery

Endoscopy & lavage

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POSSIBLE NURSING DIAGNOSES

Ineffective airway clearance r/t

tracheobronchial secretions and obstruction

Imbalanced nutrition: less than body

requirements r/t inability to digest food or

absorb nutrients

Risk for infection r/t chronic pulmonary

disease

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Planning & Interventions

Provide respiratory therapy

Administer medications Drink lots of fluids

exercise

Meet nutritional needs Provide psychosocial support

Discharge planning and home teaching