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Created: 1/6/2006
Updated: 8/1/2007

Management of anaesthesia for a teenager with cystic fibrosis

Cystic fibrosis

  • AR inherited condition: 1/2500 Caucasians
  • Chromosome 7 mutation, multiple abnormalities described
  • Expressed as defective Cl- channels
    • High Cl- concentration in secretions, increased viscosity
    • Sweat Cl- >60 mmol/L
    • Resultant respiratory, pancreatic, biliary dysfunction

Clinical problems

  • Respiratory
    • Impaired clearance of secretions, mucociliary dysfunction
    • Chronic infection, bronchiectasis, dyspnoea, excessive sputum
    • Air-trapping due to mucous plugging: COAD-like picture
    • Nasal polyps, chronic sinusitis
    • Complications
      • Respiratory failure: cyanosis, CO2 retention
      • Haemoptysis, pneumothorax, cor pulmonale
  • GIT
    • Pancreatic exocrine failure
      • Malabsorption, malnutrition without enzyme supplementation
      • Chronic pancreatitis, acute exacerbations
      • Secondary endocrine failure: diabetes mellitus
    • Bile secretion impaired
      • Fat and fat-soluble vitamin malabsorption
      • Later cirrhosis and portal hypertension
    • Neonatal meconium ileus
  • Psychological
    • Many admissions and procedures
    • Chronic illness, medicalization
    • Longer survival resulting in more adult presentations


  • Routine, plus:
  • History related to CF
    • Admissions, current therapy, respiratory disease, diabetic control
    • Previous anaesthesia
  • Examination
    • General appearance, respiratory, cardiac focus
      • Signs of respiratory failure, right heart failure
  • Investigations
    • CXR, RFT, ABG
    • FBE, U&E, LFT, glucose
    • Recent cultures of sputum


  • Consult with treating physician to achieve best respiratory function
    • Bronchodilators, saline nebulisers, antibiotics, physiotherapy


  • Regional avoids the need for intubation and risk of worsening infection
  • If GA required by nature of surgery, regional analgesia may result in better
  • respiratory function postoperatively
  • Airway
    • Humidified gases, nebulized saline may be of benefit
    • Frequent suctioning
    • Avoid nasal intubation
  • Ventilation
    • As for COAD: long I:E, minimize airway pressures, slow rate
    • Increased FiO2
    • Vigilance for pneumothorax
  • Circulation
    • Consider invasive monitoring if cor pulmonale: arterial line ± PA catheter
  • Other considerations
    • Management of diabetes: fasting, glucose monitoring
    • Choice of drugs suitable for impaired hepatic function


  • Level of care determined by severity of disease and extent of surgery
  • Active physiotherapy and early mobilization


Kindly provided by Dr James Mitchell from his pharmacodynamics series

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