G.1. Diagnosis of cystic fibrosis

Review question: In infants, children, young people and adults (including those that have undergone newborn screening) when should cystic fibrosis be suspected?

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G.2. Information and support

Review question: What information and support should be given to children, young people and adults with cystic fibrosis?

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G.3. Service delivery

G.4. Transition

Review question: What parts of the transition from children’s to adult services are most important for young people with cystic fibrosis and their family members and carers?

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G.5. Complications of cystic fibrosis

Review question: What are the non-respiratory complications of cystic fibrosis in infants, children, young people and adults?

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G.6. Pulmonary monitoring

Review question 1: What is the value of the following investigative strategies in monitoring the onset of pulmonary disease in people with CF without clinical signs or symptoms of lung disease?

• Non-invasive microbiological investigation-induced sputum samples, cough swab, throat swab, and nasopharangeal aspiration
• Invasive microbiological investigation-broncho-alveolar lavage
• Lung physiological function tests-Cardiopulmonary exercise testing, Spirometry and Lung Clearance Index
• Imaging techniques-Chest x-ray and CT scan

Review question 2: What is the value of the following investigative strategies in monitoring evolving pulmonary disease in people with established lung disease?

• Non-invasive microbiological investigation-induced sputum samples, cough swab, throat swab, and nasopharangeal aspiration
• Invasive microbiological investigation-broncho-alveolar lavage
• Lung physiological function tests-Cardiopulmonary exercise testing, Spirometry and Lung Clearance Index
• Imaging techniques-Chest x-ray and CT scan.

Review question 3: What is the added value of imaging and invasive microbiological testing in addition to non-invasive microbiological testing and lung function tests in monitoring the response to treatment following an acute exacerbation?

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G.7. Airway clearance techniques

Review question: What is the effectiveness of chest physiotherapy in people with cystic fibrosis?

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G.8. Mucoactive agents

Review question: What is the effectiveness of mucoactive or mucolytic agents, including rhDNase, nebulised saline (isotonic and hypertonic) and mannitol?

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G.9. Pulmonary infection - prophylaxis

Review question: What is the effectiveness of long-term antimicrobial prophylaxis to prevent pulmonary bacterial colonisation with Staphylococcus aureus in people with CF?

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G.10. Pulmonary infection – acute

Review question: What is the effectiveness of antimicrobial treatment for acute pulmonary infection or those with an exacerbation in children and adults with cystic fibrosis?

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G.11. Pulmonary infection – chronic

Review question: What is the effectiveness of antimicrobial regimens in suppressing chronic pulmonary infection in children and adults with cystic fibrosis with any of the following pathogens: Pseudomonas Aeruginosa, Burkholderia Cepacia Complex, Staphylococcus Aureus and Aspergillus Fumigatus?

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G.12. Immunomodulatory agents

Review question: What is the effectiveness of immunomodulatory agents in the management of lung disease, for example corticosteroids, azithromycin?

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G.13. Nurtitional interventions

Review question: What is the clinical and cost effectiveness of nutritional interventions in people with cystic fibrosis?

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G.14. Exocrine pancreatic insufficiency

Review question: In people with cystic fibrosis, what is the effectiveness of enzyme replacement therapy in the treatment of exocrine pancreatic insufficiency?

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G.15. Distal intestinal obstruction syndrome

Review question: What are the effective strategies for treatment and secondary prevention of distal ileal obstruction syndrome?

No clinical evidence was identified for this review.

G.16. Liver disease

Review question: What is the effectiveness of ultrasound scanning to detect clinically important cystic fibrosis related liver disease?

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G.17. Ursodeoxycholic acid

Review question: What is the effectiveness of ursodeoxycholic acid for preventing liver disease progression in people with cystic fibrosis?

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G.18. Cystic fibrosis related diabetes

Review question: What is the most effective strategy to monitor for the onset of CF-related diabetes (CFRD)?

No clinical evidence was identified for this review.

G.19. Bone mineral density

Review question: What is the most effective strategy to monitor for the identification of reduced bone mineral density in people with Cystic Fibrosis?

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G.20. Exercise

Review question: What is the effectiveness of programmes of exercise in the management of cystic fibrosis?

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G.21. Psychological assessment

Review question: What strategies are effective at identifying people with cystic fibrosis for the presence of a psychological and/or behavioural problem?

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G.22. Prevention of Cross-infection

Review question 1: What is the effectiveness of cohorting on the basis of pathogen status versus not cohorting on the basis of pathogen status in reducing transmission of CF pathogens?

Review question 2: What is the effectiveness of different models of segregating patients in reducing transmission of CF pathogens?

Review question 3: What is the effectiveness of individual protective equipment in reducing transmission of CF pathogens?

Review question 4: What is the effectiveness of the combination of cohorting, segregating and protective equipment in reducing transmission of CF pathogens?

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