Inherited disorders: Cystic fibrosis
Cystic fibrosis (CF) is a disorder usually diagnosed in childhood in which a genetic mutation disrupts the movement of chloride ions and water across membranes. This means that secretions in certain parts of the body such as the lungs, pancreas and gut, become very thick and are difficult to clear. CF is a complex condition, and we offer only a very basic introduction here.
Chronic respiratory complications
Mucous accumulates in the lungs which becomes infected by bacteria (most commonly Pseudomonas aeruginosa). Recurrent, intermittent infections occur and can become chronic, which may accelerate a decline in respiratory function. Most people who die of CF each year are young adults, and they typically die from lung-related causes. So preventing chronic chest infection is a key element in increasing survival. Airways clearance techniques such as physiotherapy help to reverse the build up of mucous, and medicines such as inhaled dornase alfa or hypertonic saline reduce the viscosity of lung secretions. Acute infections are treated with inhaled antibiotics such as tobramycin or colistin, and oral azithromycin has been given as a long-term oral prophylaxis.Gastrointestinal complications

Cystic fibrosis and the pharmacist
![]() |
© Crown copyright 2017 |
Patients with CF may need medicines that are not commonly used in other patient groups, and so pharmacists have a particular role to ensure that prescribed regimens are safe and correct. The medicines concerned may be unlicensed, may be given via an unlicensed route, or be taken at larger than expected doses. Hospital pharmacists can show patients how to administer medicines, and help them with adherence and monitoring of therapy. You can also organise communications about ongoing supplies of medicines by providing appropriate information to primary care colleagues such as GPs and community pharmacists.
Specialist cystic fibrosis pharmacists are experts at medicines optimisation in this group of patients, so you should try to contact one for advice before making a significant intervention.