Management of Cystic Fibrosis

Lung Management

Lung infections are a hallmark of CF and are also the leading cause of CF death. The disease is punctuated by exacerbations followed by improvement with intensive therapy. As the disease progresses, exacerbations recur with greater frequency and resulting complications.

The goals of management are maintenance of airway clearance, prevention of bacterial exacerbations and inflammation, and maintenance of nutritional support. On regular visits, patients may receive the following:

  • Assessment of symptoms
  • Physical examination
  • Assessment of lung function by spirometry and oxygen saturation
  • Respiratory therapy or physiotherapy for lung management

It is recommended that patients have sputum assessments to identify pathogens and assess antibiotic susceptibility at least once per year.18,27

References:
18. Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest 2004 January;125(1 Suppl):1S-39S.
27. Centers for Disease Control and Prevention. Newborn Screening for Cystic Fibrosis. Atlanta, GA: U.S Department of Health and Human Services; 2004 Oct 15. Report No.: MMWR/53/RR-13.

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