Table 1

Common cystic fibrosis related pathologies, complications and principal elements of management in intensive care (for details see text)

CF related pathologyComplicationManagement
Lung disease
    Bacterial colonisation of airwaysInfective exacerbations• Antibiotics
• Physiotherapy
• DNase
Multiresistant pathogens• Antibiotics selected according to sensitivities
Sputum plugging• Endobronchial suction
• Bronchoscopy
• DNase
    Obstructive lung diseaseDifficult ventilation• Bronchodilators
Prolonged weaning• Tracheostomy
• NIV
    OtherHaemoptysis• Tranexamic acid
• Bronchial artery embolisation
Gastrointestinal diseasePancreatic insufficiency• Pancreatic enzymes
• Elemental feed
Distal intestinal obstruction syndrome• Maintain good hydration
• Lactulose
• N-acetyl cysteine
• Gastrografin enema
Liver diseaseCoagulopathy• Vitamin K
• Fresh frozen plasma
Thrombocytopenia• Platelet transfusion
Altered drug metabolism• Careful drug prescribing practice
Variceal haemorrhage• Variceal banding
DiabetesHyperglycaemia• Intravenous insulin
Other
    CF upper airway diseaseSinusitis• Avoid nasal intubation
• Antibiotics