Cystic Fibrosis

EDEN BETH B. ESPINO

BSN3-A
USA-CN

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 transport
of chloride and sodium across an epithelium, leading to
thick, viscous secretions.

PATHOPHYSIOLOGY

Predisposing
factors:
heridity
age
gender
family history
race

Mutation in the gene of Cystic

fibrosis transmembrane
conductance regulator (CFTR)

Body produces thick, sticky

mucus and it will clogs the lungs
thus causing stagnant mucus

infected cells creates problems

in the respiratory and digestive
tracts
Obstructs the
pancreas

Malabsorption &
malnutrition

Cystic fibrosis

Precipitating
factors:
smoking
lung infections
electrolyte
imbalance
Diabetes
pulmonary
hypertension

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

Diagnostic Tests

Sweat Test
Genetic Analysis
Organ function tests
Sputum culture
Lung function tests
Imaging tests

MEDICAL MANAGEMENT

Antibiotics
Mucus-thinning drugs
Bronchodilators
Pain relievers
Oral pancreatic enzymes and better nutrition
Breathing devices
Oxygen therapy

SURGICAL MANAGEMENT

Lung transplant
Bronchial airway drainage
Nasal polyp removal
Bowel surgery
Endoscopy & lavage

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

Planning & Interventions

Provide respiratory therapy

Administer medications
Drink lots of fluids
exercise
Meet nutritional needs
Provide psychosocial support
Discharge planning and home teaching