Importance of controlling mucus in the cycle of cystic fibrosis (CF) with a multitherapy approach
DNA-laden mucus leads to a cycle of obstruction, infection, and inflammation 2
Controlling mucus is an essential component in the management of CF.
The buildup of material called extracellular DNA makes the mucus in the lungs even thicker than before, making it more difficult to clear the lungs properly.
As mucus continues to get thicker, more bacteria become trapped, which can eventually lead to a respiratory tract infection.
As white blood cells attempt to fight the germs caught in the mucus, they leave behind extracellular DNA. The buildup of these remains leads to further obstruction and lung damage.
CFTR=cystic fibrosis transmembrane conductance regulator.
See why mucus is now considered a key driver of CF progression 2,4
Even prior to the onset of CF symptoms, the mucus cycle can lead to
progression of lung disease and irreversible damage. Consequently,
early clearing and thinning of DNA-laden mucus can be important to
help reduce lung damage.
Fighting CF often requires a multitherapy approach 6
Every prescribed therapy has an important role in managing the destructive cycle of CF by targeting different disease mechanisms. Failure to interrupt the obstruction-infection-inflammation cycle eventually leads to lung damage and respiratory failure. 7,8
|A multitherapy approach targets different disease mechanisms|
|Pulmozyme (dornase alfa) can help reduce mucus viscosity by cleaving extracellular DNA, and breaking up sticky mucus 11,12|
|Improve the chloride ion conduction by modulating the defective CFTR channel protein 9,10|
|Restore airway surface liquid that has been depleted 11|
AIRWAY CLEARANCE TECHNIQUES
|Move mucus through the airways due to defective mucociliary clearance 13,14|
|Kill bacteria and help prevent and treat infection 11,12|
|Open the airways more by relaxing the inflamed airway lining due to structural damage/bronchiectasis 15|
The distinct MOA of Pulmozyme targets the DNA in CF mucus
Important Safety Information
Pulmozyme is contraindicated in patients with known hypersensitivity to dornase alfa, Chinese Hamster Ovary cell products, or any component of the product.
The most common adverse reactions associated with the use of Pulmozyme include: voice alteration, pharyngitis, rash, laryngitis, chest pain, conjunctivitis, rhinitis, decrease in FVC of ≥ 10%, fever, dyspepsia, and dyspnea. There have been no reports of anaphylaxis attributed to the administration of Pulmozyme. Mild to moderate urticaria and mild skin rash have been observed and have been transient.
Pulmozyme (dornase alfa) is indicated for daily administration in conjunction with standard therapies for the management of cystic fibrosis (CF) patients to improve pulmonary function.
In CF 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.
The safety and effectiveness of Pulmozyme have been established in pediatric patients 5 years of age and older. The safety of Pulmozyme, 2.5 mg by inhalation, was studied with 2 weeks of daily administration in 65 patients with cystic fibrosis aged 3 months to < 5 years. While clinical trial data are limited in pediatric patients younger than 5 years of age, the use of Pulmozyme should be considered for pediatric CF patients who may experience potential benefit in pulmonary function or who may be at risk of respiratory tract infection.
The safety of Pulmozyme, 2.5 mg by inhalation, was studied with 2 weeks of daily administration in 98 pediatric patients with cystic fibrosis 3 months to 10 years of age (65 aged 3 months to < 5 years, 33 aged 5 to ≤ 10 years). The PARI BABY™ reusable nebulizer (which uses a facemask instead of a mouthpiece) was utilized in patients unable to demonstrate the ability to inhale or exhale orally throughout the entire treatment period (54/65, 83% of the younger; and 2/33, 6% of the older patients). Overall, the nature of adverse reactions was similar to that seen in the placebo-controlled trials in older patients. The number of patients reporting cough was higher in the younger age group as compared to the older age group (29/65, 45%; compared to 10/33, 30%) as was the number reporting moderate to severe cough (24/65, 37%; compared to 6/33, 18%). The number of patients reporting rhinitis was higher in the younger age group as compared to the older age group (23/65, 35%; compared to 9/33, 27%) as was the number reporting rash (4/65, 6% as compared to 0/33, 0%).