Cystic Fibrosis Cycle and Pulmozyme

Controlling mucus is an essential component in the management of cystic fibrosis 7

DNA-laden mucus leads to a cycle of obstruction, infection, and inflammation 8-10

cycle diagram
Failure to the interrupt

Pulmozyme is the only EMA-approved mucolytic that breaks up and thins mucus via DNase activity1, 13-14

extracellular
  • The build-up of neutrophil-derived DNA in the mucus of patients with cystic fibrosis makes it thicker and more difficult to clear from the lungs properly 6
  • Pulmozyme is a genetically engineered version of a naturally occurring recombinant human DNase 1
  • Pulmozyme decreases mucus viscosity by cleaving the extracellular DNA 1
reduction in cystic fibrosis

1. Pulmozyme SmPC, Electronic Medicines Compendium, accessed 10/8/18; 

3. Harms  HK et al. Dnase International Study Group. Multicenter, open-label study of recombinant human Dnase in cystic fibrosis patients with moderate lung disease. Pediatr Pulmonol. 1998; 23(3): 155-161; 

4. King, M. Mucolytics and mucus clearance. In Rubin BK, van der Sxhans CP, eds. Therapy for Mucus-Clearance Disorders. New Yoirk, NY: Marcel Dekker Inc, 2004:201-224; 

5. Chmiel, JF, Kanstan MW. Anti-inflammatory medication for cystic fibrosis lung disease: selecting the most appropriate agent. Treat Respir Med. 2005; 4(4):255-273; 

6. Puchelle E et al. Physical and functional properties of airway secretions in cystic fibrosis – therapeutic approaches. Respiration. 1995; 62(suppl 1) 2-12; 

7. Lyczak, JB et al. Lung infections associated with cystic fibrosis. Clin Microbiol Rev. 2—2; 15(2):194-222; 

8. Ratjen FA. Cystic fibrosis: pathogenesis and future treatment strategies. Respir Care. 2009; 54(5): 595-605; 

9. Chmiel JF et al. The role of inflammation in the pathophysiology of CF lung disease. Clin Rev Allergy Immunol. 2002; 23(1):5-27.;

10. Shak, S. Aerosolized recombinant human Dnase I for the treatment of cystic fibrosis. Chest. 1995; 107(2 Suppl): 65S-70S. 

11. Flume PA & Van Devanter DR. State of progress in treating cycstic fibrosis respiratory disease. BMC med. 2012:10:88; 

12. Mogayzel PJ et al and Pulmonary Clinical Proactice Gidelines Committee. Cystic Fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013;187(7):680-689.

13. Bakker EM et al. Small airway deposition of dornase alfa during exacerbations in cystic fibrosis; a randomized controlled clinical trial. Pediatr Pulmonol. 2014; 49(2): 154-161; 

14. Smyth AR et al. European Cystic Fibrosis Society standards of care; best practice guidelines. J Cyst Fibros. 2014; (13 Suppl 1):S23-42.