Principles of managing cystic fibrosis

This topic is not a comprehensive guide to the management of cystic fibrosis (CF). All patients with CF in Australia are managed by, or in consultation with, a multidisciplinary specialist CF centre. This has been shown to improve clinical outcomes. Treatment protocols vary among different specialist CF centres. Changes in management should only be made after consultation with the specialist CF centre. If a patient with CF is admitted to hospital, contact the patient’s specialist CF centre.

For patients with CF, inflammation and infection of the airways occur early in infancy, leading to structural changes of bronchiectasis that progress with age. Progressive lung disease is the major cause of morbidity and mortality in CF; aggressive management of lung disease is vital to limit progression.

The treatment modalities in CF include:

• effective airway clearance, using physical techniques and drug treatment (eg mucolytic agents)
• anti-inflammatory treatment
• drug treatment to correct cystic fibrosis transmembrane conductance regulator (CFTR) protein function in patients with particular mutations of the CF gene; see CFTR modulator therapy for CF
• early aggressive antibiotic therapy
• strict infection-control measures
• managing nonrespiratory aspects (eg nutrition, gastrointestinal aspects, cystic fibrosis–related diabetes, fertility, bone health, kidney disease, mental health).

Adherence to treatment is an important consideration in the management of CF; management can include complex, time-consuming and expensive treatment regimens. Keeping management as simple as possible, including minimising time-consuming treatments such as nebulised drugs, helps to reduce barriers to adherence. Involving the patient in treatment decisions, including in development of their management plan, can also improve adherence.

Exacerbations and declines in lung function can still occur despite good adherence to treatment; this can be discouraging to patients. Education about CF can prepare the patient for setbacks and encourage them to maintain adherence. Encourage the patient to ask questions about their disease and its management.

Asthma may coexist with CF. Asthma is difficult to diagnose in patients with CF because many of the respiratory symptoms and lung function test findings are similar. Treat asthma in a patient with CF as for any patient with asthma. See Asthma maintenance management in adults and adolescents and Asthma maintenance management in children.

The Cystic Fibrosis Australia website has information brochures on various CF treatments for health professionals, patients, parents and carers.