Cystic Fibrosis

Define Cystic Fibrosis?

Genetic disorder affecting the lungs, pancreas, liver, intestine and reproductive organs

• Main symptoms pulmonary disease → Viscous sputum, chest infections and malabsorption

What Mucoactive Agents are used?

Dornase alfa → first choice
hypertonic sodium chloride

What CTFR Modulators are used?

Ivacaftor - tezacaftor - elexacaftor combination

What antibiotics are often used?

Staph A (Flucloxacillin)
PO if well

IV if not

• Pseudomonas aeruginosa

  • PO/IV + inhaled antibacterial inhalation

Nebulised colistimethate sodium 

Immunomodulatory Agents:

Offer long term azithromycin

offered to patients with deteriorating lung functions or repeated exacerbations

Exocrine Pancreatic Insufficiency:

Pancreatic enzyme replacement therapy

H2 or PPI can be considered for patients who have persistent symptoms or signs of malabsorption → acid suppressive

What else needs to be monitored in cystic fibrosis?

Monitor intestinal obstruction, liver disease, diabetes and bone mineral density

Non drug treatment

• Specialist physiotherapists

• The importance of airway clearance techniques should be discussed

How often should drug treatment be reviewed?

Review at least every 3 months