management of pulmonary disease

Last reviewed 06/2021

Treatment of respiratory disease is started early and aimed at prevention.

This is directed towards:

• bronchial clearance, in particular by vigorous daily physiotherapy, with manual percussion, forced expiratory manoeuvres and vibration in a variety of positions. There may also be a role for postural drainage - lying in a variety of positions
  
  
• antibiotic treatment can be prophylactic and resposive.
• often lifelong to protect against Staph. aureus, the commonest childhood pathogen, Haemophilus inflenzae and Pseudomonas aeruginosa
• antibiotics may be intravenous, using a Port-a-Cath, or they may be nebulised and inhaled
• Pseudomonas becomes a major pathogen with increasing age
• Pseudomonas cepacia colonisation may be associated with accelerated lung disease
• Burkholderia cepacia complex (1)
  • for people with cystic fibrosis who develop a new Burkholderia cepacia complex infection (that is, recent respiratory sample cultures showed no Burkholderia cepacia infection):
    • whether they are clinically well or not, give antibiotic eradication therapy using a combination of intravenous antibiotics
    • seek specialist microbiological advice on the choice of antibiotics to use.
      
      
• immunomodulatory agents (1)
  • if deteriorating lung function or repeated pulmonary exacerbations, offer long-term treatment with azithromycin at an immunomodulatory dose
  • if continued deterioration in lung function, or continuing pulmonary exacerbations while receiving long-term treatment with azithromycin, stop azithromycin and consider oral corticosteroids
  • do not offer inhaled corticosteroids as an immunomodulatory treatment for cystic fibrosis per se
    
    
• bronchodilators and steroids (oral or inhaled) may be beneficial in patients with allergic bronchopulmonary aspergillosis
  
  
• mucolytics (DNA-ase) improve mucus expectoration
  
  
• mannitol dry powder for inhalation is recommended as an option for treating cystic fibrosis in adults (2)
  
  
• exercise - anecdotal evidence that vigorous aerobic exercise slows lung deterioration; it is said to improve bronchial clearance
  
  
• heart-lung transplantation is a final possibility

Reference:

• 1) NICE (October 2017) Cystic Fibrosis.
• 2) NICE (November 2012). Mannitol dry powder for inhalation for treating cystic fibrosis