From the Guidelines
Bronchitol (mannitol) and Pulmozyme (dornase alfa) are both essential medications for improving lung function in patients with cystic fibrosis, with Pulmozyme being strongly recommended for patients 6 years of age and older with moderate to severe lung disease, as stated by the Cystic Fibrosis Foundation 1.
Mechanism of Action
- Bronchitol is an inhaled dry powder that creates an osmotic gradient in the airways, drawing water into the mucus layer and making it less viscous and easier to clear.
- Pulmozyme contains recombinant human DNase, an enzyme that breaks down DNA released by neutrophils in the thick mucus of CF patients, reducing mucus viscosity and improving clearance.
Administration and Dosage
- Bronchitol is typically administered at a dose of 400 mg (10 capsules of 40 mg) twice daily via a special inhaler, with patients required to pass a tolerance test to ensure they don't experience bronchospasm.
- Pulmozyme is administered as a 2.5 mg nebulized solution once daily.
Efficacy and Safety
- Both medications help address the fundamental problem in CF—thick, sticky mucus that obstructs airways and harbors infections—but through complementary mechanisms.
- They can be used together as part of a comprehensive airway clearance regimen that typically includes physical techniques and other medications.
- Side effects for Bronchitol may include cough and bronchospasm, while Pulmozyme is generally well-tolerated with occasional voice alterations or rash.
- The Cystic Fibrosis Foundation strongly recommends the chronic use of dornase alfa to improve lung function and reduce exacerbations in patients with CF, 6 years of age and older, with moderate to severe lung disease 1.
- The use of Pulmozyme has been shown to improve lung function, quality of life, and reduce exacerbations in patients with CF, as demonstrated in various studies 1.
From the FDA Drug Label
12 CLINICAL PHARMACOLOGY
- 1 Mechanism of Action PULMOZYME is recombinant human deoxyribonuclease I (rhDNase), an enzyme which selectively cleaves DNA. In preclinical in vitro studies, PULMOZYME hydrolyzes the DNA in sputum of CF patients and reduces sputum viscoelasticity. In CF patients, retention of viscous purulent secretions in the airways contributes both to reduced pulmonary function and to exacerbations of infection Purulent pulmonary secretions contain very high concentrations of extracellular DNA released by degenerating leukocytes that accumulate in response to infection.
The function of Pulmozyme (dornase alfa) in patients with cystic fibrosis is to:
- Hydrolyze the DNA in sputum
- Reduce sputum viscoelasticity
- Improve pulmonary function
- Reduce the risk of respiratory tract infections requiring parenteral antibiotics
There is no information in the provided drug labels about Bronchitol (mannitol). 2 2 2
From the Research
Function of Bronchitol (Mannitol) and Pulmozyme (Dornase Alfa) in Cystic Fibrosis
- Bronchitol (mannitol) is a hyperosmolar agent that increases the airway surface liquid volume and enhances mucociliary clearance in patients with cystic fibrosis 3, 4, 5.
- Pulmozyme (dornase alfa) is a mucolytic agent that reduces mucus viscosity by cleaving the DNA released from neutrophils, thereby improving clearance of secretions and preventing airway infections and damage to the lung parenchyma 3, 6, 7.
Mechanism of Action
- Mannitol works by changing the viscoelastic properties of airway secretions, increasing the hydration of the airway surface liquid, and contributing to increased mucociliary and cough clearance of retained secretions 4, 5.
- Dornase alfa cleaves the DNA released from neutrophils, reducing mucous viscosity and promoting improved clearance of secretions 6, 7.
Clinical Benefits
- Both mannitol and dornase alfa have been shown to improve lung function and reduce pulmonary exacerbations in patients with cystic fibrosis 3, 6, 4, 5.
- Dornase alfa has been found to improve forced expiratory volume at one second (FEV1) and reduce the number of pulmonary exacerbations in trials of up to two years 6.
- Mannitol has been shown to increase FEV1 and FVC in a dose-dependent manner, with the 400mg dose having the greatest improvement 4.