Management

The underlying principles of management of cystic fibrosis are:

• optimisation of physical and psychological growth
• delaying the progress of the lung disorder
• making the lifestyle of the patient and their family as normal as possible.

Thus, there are measures to promote bronchial clearance and control pulmonary infection, measures to support nutrition, counselling and emotional support.

Medicines for lung problems:

• antibiotics
• mannitol dry powder for inhalation for treating CF
• colistimethate sodium and tobramycin dry powders for inhalation for treating Pseudomonas lung infection in CF
• Nasal polyps may be treated with topical steroids which may cause the polyps to shrink but do not eradicate them.

Airway clearance techniques such as active cycle of breathing techniques (ACBT) or use of airway clearance devices.

Regular exercise improves both lung function and overall fitness.

Patients with cystic fibrosis may be candidates for a heart-lung transplant operation.

Reference:

• (1) NICE (October 2017) Cystic Fibrosis. Available at https://www.nice.org.uk/guidance/ng78. (Accessed on 05 June 2022).
• (2) NHS (2021) Cystic Fibrosis. Available at https://www.nhs.uk/conditions/cystic-fibrosis/. (Accessed on 05 June 2022).