22nd February 2024

Management

Cystic fibrosis management incorporates an array of different strategies. These include conservative management, disease education, regular physiotherapy, antibiotic therapies for exacerbation to direct CFTR modulator therapy and lung transplantation.

CF patients should have an annual pneumococcal, seasonal flu and COVID 19 vaccination to reduce the risk of viral exacerbation. Regular chest physiotherapy, including postural drainage, helps with mucus expectoration. Regular prescription of beta 2 agonists and inhaled corticosteroids provide symptomatic relief and reduce the propensity for future exacerbations. Regular antibiotic therapy such as azithromycin has been shown to be effective against particular bacterial colonisation such as Pseudomonas Aeruginosa. 

Regular input from multidisciplinary teams is essential in the management of CF patients. Dietitians work closely to reduce growth impedance by addressing nutritional deficiencies due to secondary organ involvement, i.e. pancreatic insufficiency, reduced gut motility, etc. Physiotherapists and occupational therapists work closely to ensure mobility optimisation and aids which help with adaptation at home. 

Psychologist and psychiatry input is paramount as CF patients are oftentimes young with chronic long term sequalae, affecting multiple organs and reducing the negative impact from a psychological perspective. 

Patients with severe disease are considered for the lung/liver transplantation register provided there are no significant contraindications such as solid organ/ haematological malignancies, raised BMI (>35), advanced dysfunction of any other major, lifestyle risk factors including excessive alcohol consumption, smoking or use of illicit substances or non-compliance with medication therapies. Relative contraindication considers age above 60, end organ damage including chronic kidney disease, heart failure, diabetes and need for mechanical ventilation support.  Overall outcomes for lung/liver transplantation are encouraging with 5-year survival >60% and 10-year survival >50%.

References

• Meachery G, De Soyza A, Nicholson A, et al. Outcomes of lung transplantation for cystic fibrosis in a large UK cohort Thorax 2008;63:725-731.
• Weill D. Lung transplantation: indications and contraindications. J Thorac Dis. 2018 Jul;10(7):4574-4587. doi: 10.21037/jtd.2018.06.141.