Medical management of CF

• Medical management – respiratory:

  • Bronchodilators (if appropriate)
  • Steroids (if appropriate)
  • Antibiotics (oral or IV) during an exacerbation
  • How is the correct antibiotic chosen for the patient? - checking what antibiotics work against the microbes in sputum sample
  • Nebulised antibiotics (if colonised with pseudomonas)
  • Nebulised Pulmozyme (DNase) – mucolytic - breaks down sputum (makes it more runny)
  • Nebulised hypertonic saline
  • Inhaled Mannitol
• Portacaths:

  • Useful for patients who require regular courses of injected antibiotics
  • Caution with manual techniques
• Medical management – respiratory:

  • Long term oxygen therapy
  • Non invasive ventilation (bridge to transplant)
  • Lung transplants
  • lack of suitable donor organs
  • one third of individuals on the transplant list will die waiting for a donor organ
• Other medical management:

  • Dietician
  • Vitamin supplements
  • Creon (pancreatic enzymes)
  • Gastrostomy feeding
  • Diabetes management
  • Liver transplant