Cystic Fibrosis (CF)

Question 1

CF pathophysiology

Answer 1

Obstruction of ducts of the exocrine system is caused by thick, viscous secretions – these adhere to lumen of the ducts which eventually undergo fibrosis

Thick secretions obstruct bronchioles, leading to air trapping and hyperinflation.

The hallmark of respiratory involvement in CF is its effect on the airways. The disease progresses from being a disease of the small airways (chronic bronchiolitis) to involvement of the larger airways, and finally causes destruction of lung tissue.

Death usually results from loss of pulmonary function.

Exocrine function of pancreas is altered or may be lost completely.

Question 2

Symptoms

Answer 2

Early manifestations 
Failure to grow
Digital clubbing
Persistent cough with mucous production
Tachypnea
Large, frequent bowel movements
Large, protuberant abdomen with emaciated appearance of extremities

First symptom in adult is usually frequent cough.
Becomes viscous and purulent
Produces greenish-coloured sputum

As disease progresses
Exacerbations of ↑ cough 
Weight loss
↑ Sputum
↓ In pulmonary function

Exacerbations become more frequent, and recovery of lost lung function is less complete.

Question 3

Complications of CF

Answer 3

Pneumothorax is common (>10% of clients)
Hemoptysis (can be life-threatening)
Digital clubbing
Respiratory failure – late complication
Cor pulmonale – late complication

Question 4

CF Diagnosis

Answer 4

Sweat test + Genetic Testing

• Even though the sweat chloride test is the gold standard for CF diagnosis, the diagnosis is not clear cut in all individuals, especially in adults.
• A second sweat chloride test is recommended to confirm the diagnosis unless genetic testing identifies two CF mutations.
• In genetic testing, a blood sample or cells taken from the inside of the cheek are sent to a laboratory that specializes in genetic testing. Most labs test only for the most common mutations of the CF gene.
• A genetic test is often used if the results from a sweat test are unclear.

Question 5

Collaborative Care

Answer 5

• Promote clearance of secretions.
• Control infection in the lungs.
• Provide adequate nutrition.
• Manage pulmonary problems (e.g., relieving airway obstruction).
• Aerosol and nebulization treatments of medications to liquefy mucus and to facilitate coughing
• Airway clearance techniques
• CPT
• Aerobic exercise can be effective in clearing airways.
• Early intervention with antibiotics when lungs become infected
• Sclerosing indicated for recurrent pneumothorax
• Lung transplants
• Replacement of pancreatic enzymes and supplements

Question 6

Nursing implementation

Answer 6

Respiratory intervention for the client with CF targets relief of bronchoconstriction, airway obstruction, and airflow limitation through the use of aggressive chest physiotherapy, antibiotics, and bronchodilators.

Home management: an aggressive plan of postural drainage with percussion and vibration, aerosol nebulization therapy, and breathing retraining