How normal mucus helps protect the airways

In healthy lungs, the physical properties of normal mucus enable mucus transport via cough and ciliary beating, which protect the airways.
How cystic fibrosis affects mucus
In patients with cystic fibrosis, DNA-laden mucus contributes to a downward spiral of obstruction, inflammation and infection leading to lung damage (Ref. 1, 2, 3). Abnormal ion exchange across the epithelial layer in the lungs causes this thick, adhesive mucus to form, eventually reducing mucociliary clearance and trapping pathogens (Ref. 1, 2, 3, 4, 5). Controlling mucus is an essential part of cystic fibrosis management (Ref. 6).
Read: Pulmozyme® Interactive Brochure
Our new interactive brochure puts the latest information on CF at your fingertips. Explore the unique Pulmozyme MOA with infographics, supportive text and clinical information. You can also review epidemiological data and information on the pathophysiology of cystic fibrosis, as well as other current and potential CF therapies, including mucus alterators, anti-infectives, hydrators and CFTR modulators.
Watch: KOL Roundtable Video
In this overview, Dr. Richard Moss, Professor of Pediatrics at Stanford University, leads a discussion on the latest thinking about CF, treatments and patient outcomes. Moss and his colleagues highlight the role of inflammation in the pathophysiology of CF, new trends in treatment, the variability in treatment across centers, and also provide guidelines for up-to-date practice.
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- Puchelle E, de Bentzmann S, Zahm JM. Physical and functional properties of airway secretions in cystic fibrosis-therapeutic approaches. Respiration. 1995;62(suppl 1):2-12. P 6, col 1, par 1, L16-22.
- Boucher RC. New concepts of the pathogenesis of cystic fibrosis lung disease. Eur Respir J. 2004;23:146-158. P 153, col 1, par 3, L3-5; par 5, L1-3.
- Shak S. Aerosolized recombinant human DNase I for the treatment of cystic fibrosis. Chest. 1995;65S-70S. P 65S, col 1, par 1, L5-8; par 2, L1-7.
- Coakley RD, Boucher RC. Pathophysiology: epithelial cell biology and ion channel function in the lung, sweat gland and pancreas. In: Hodson M, Geddes D, Bush A, eds. Cystic Fibrosis. 3rd ed. London, UK: Hodder Arnold; 2007:59-68. P 62, col 1, par 3, L15-21.
- Shak S, Capon DJ, Hellmiss R, Marsters SA, Baker CL. Recombinant human DNase I reduces the viscosity of cystic fibrosis sputum. Proc Natl Acad Sci. 1990;87:9188-9192. P 9188, col 1, par 2, L1-8.
- Cystic Fibrosis Foundation. CF Care Guidelines - Respiratory. http://www.cff.org/treatments/CFCareGuidelines/Respiratory/. Accessed August 17, 2010. P 1, par 1-3.