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Abnormal Mucus and Disease Progression

In the earliest stages of CF, abnormal mucus contributes to an unhealthy environment in the lungs

Normal airway mucus has beneficial properties that help protect the airways

Normal mucus can be easily transported

The physical properties of normal mucus enable mucus transport via cough and ciliary beating, which protect the airways

From birth, CF impairs the beneficial properties and protective function of airway mucus

Abnormal mucus slows transport and leads to obstruction

  • A chronic, exaggerated, neutrophil-dominated inflammatory response is present early in CF lungs (Ref: 6-9)
  • DNA left behind by ongoing neutrophilic death increases the viscosity and adhesive properties of mucus (Ref: 2,4,10)
    • Extracellular DNA levels can be 4-fold higher in the bronchoalveolar lavage (BAL) fluid of infants with CF vs non-CF infants (Ref: 8)

Airway obstruction contributes to a cycle of persistent infection, inflammation, and additional obstruction

Tenacious mucus is a key contributor to the downward progression of CF lung disease

The downward spiral of CF lung disease

Highly adhesive mucus contributes to a downward spiral of disease and lung function decline.

The objective of early identification and intervention is to reduce risk and minimize structural lung damage

The downward spiral of CF lung disease

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References

  1. Houtmeyers E et al. Eur Respir J. 1999;13(5):1177-1188.
  2. Puchelle E et al. Respiration. 1995;62(suppl 1):2-12.
  3. Rogers DF. Therapy for Mucus-Clearance Disorders. New York, NY: Marcel Dekker Inc; 2004:1-27.
  4. King M. In: Rubin BK, van der Schans CP, eds. Therapy for Mucus-Clearance Disorders. New York, NY: Marcel Dekker Inc; 2004:201-224.
  5. Boucher RC. Eur Respir J. 2004;23(1):146-158.
  6. Chmiel JF, Konstan MW. Treat Respir Med. 2005;4(4):255-273.
  7. Khan TZ et al. Am J Respir Crit Care Med. 1995;151(4):1075-1082.
  8. Kirchner KK et al. Am J Respir Crit Care Med. 1996;154(5):1426-1429.
  9. Ratjen F et al. Pediatr Pulmonol. 2005;39(1):1-4.
  10. Shak S et al. Proc Natl Acad Sci U S A. 1990;87(23):9188-9192.
  11. Harms HK et al. Pediatr Pulmonol. 1998;26(3):155-161.

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