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Indication Statement:

Daily administration of Pulmozyme (dornase alfa) in conjunction with standard therapies is indicated in the management of cystic fibrosis (CF) patients to improve pulmonary function. In patients with an FVC ≥ 40% of predicted, daily administration of Pulmozyme has also been shown to reduce the risk of respiratory tract infections requiring parenteral antibiotics. Safety and efficacy of daily administration have not been demonstrated in patients for longer than twelve months.

Important Safety Information

  • Pulmozyme should not be used in patients with known hypersensitivity to dornase alfa, or any ingredients of the product.
  • Pulmozyme should be used in conjunction with standard therapies for CF.
  • Most common reported adverse events associated with the use of Pulmozyme include: voice alteration, pharyngitis, laryngitis, rash, chest pain, and conjunctivitis.

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How Cystic Fibrosis Progresses

About Cystic Fibrosis

Abnormal mucus slows transport and leads to obstruction

In patients with cystic fibrosis, the disease is present from birth. In a clinical study, radiological evidence of structural lung disease was common among infants with CF, 81% (46/57) of whom had abnormal computed tomography (CT). Among these subjects, 19% had bronchial dilation, 45% had bronchial wall thickening, and 67% had gas trapping (median age 3.6 months) (Ref. 1). There is also evidence of an exaggerated inflammatory response occurring early in CF, even in the absence of positive bacterial cultures (Ref. 2, 3).

A progression of obstruction and inflammation

In the lungs of CF patients, abnormal ion exchange across the epithelial layer results in thick, adhesive mucus (Ref. 4, 5). Neutrophils recruited to address lung inflammation release DNA upon necrosis that further increases viscosity and adhesiveness of mucus (Ref. 6, 7). This abnormal mucus can reduce mucociliary clearance and eventually trap pathogens, contributing to infection (Ref. 8, 9, 10, 11). Failing to interrupt the effects of this abnormal mucus on airways may eventually lead to loss of lung function. (Ref. 12, 13). The leading cause of death in CF is lung failure (Ref. 14).

Cystic fibrosis progression spiral

Mucus control - an essential part of CF management

Mucus control and cystic fibrosis management

References
  1. Sly PD, Brennan S, Gangell C, et al, on behalf of AREST CF. Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening. Am J Respir Crit Care Med. 2009;180:148- 149.
  2. Khan TZ, Wagener JS, Bost T, et al. Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med. 1995;151:1079.
  3. Armstrong DS, Hook SM, Jamsen KM, et al. Lower airway inflammation in infants with cystic fibrosis detected by newborn screening. Pediatr Pulmonol. 2005;40:503-504.
  4. Boucher RC. New concepts of the pathogenesis of cystic fibrosis lung disease. Eur Respir J. 2004;23:150.
  5. 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; 2007:62.
  6. Chmiel JF, Konstan MW. Anti-inflammatory medications for cystic fibrosis lung disease: selecting the most appropriate agent. Treat Respir Med. 2005;4:256.
  7. King M. Mucolytics and mucus clearance. In: Rubin BK, van der Schans CP, eds. Therapy for Mucus-Clearance Disorders. New York, NY: Marcel Dekker Inc; 2004:201-224.
  8. Puchelle E, de Bentzmann S, Zahm JM. Physical and functional properties of airway secretions in cystic fibrosis-therapeutic approaches. Respiration. 1995;62(suppl 1):6.
  9. Boucher RC. New concepts of the pathogenesis of cystic fibrosis lung disease. Eur Respir J. 2004;23:153.
  10. Shak S. Aerosolized recombinant human DNase I for the treatment of cystic fibrosis. Chest. 1995;65S.
  11. 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.
  12. Tiddens HA. Detecting early structural lung damage in cystic fibrosis. Pediatr Pulmonol. 2002;34:229.
  13. Boucher RC. New concepts of the pathogenesis of cystic fibrosis lung disease. Eur Respir J. 2004;23:152.
  14. Liou TG, Adler FR, Cox DR, Cahill BC. Lung transplantation and survival in children with cystic fibrosis. N Engl J Med. 2007;57:2144.
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