Pulmozyme is FDA-approved for once- or twice-daily dosing 

Instructions for dosing and administering Pulmozyme in cystic fibrosis (CF) patients

Pulmozyme is FDA-approved for once- or twice-daily administration. The recommended dose for most patients with CF is 2.5 mg once daily (QD); however, some patients over 21 years of age may benefit from 2.5 mg twice-daily (BID) dosing to reduce exacerbation risk. 1

Your patients and/or their caregivers will likely have questions about how to administer Pulmozyme. Each Pulmozyme treatment, dosed QD or BID, requires the following items 1:

  • One ampule of Pulmozyme
    • Pulmozyme is supplied in single-use ampules. It should be stored under refrigeration and protected from strong light
  • One jet nebulizer approved for use with Pulmozyme that can be connected to an air compressor OR a nebulizer system
  • Tubing and other components that come with the nebulizer
  • A mouthpiece or a face mask (for younger patients unable to inhale or exhale consistently using a mouthpiece)

Remember to communicate these important instructions to patients/caregivers:

  • Do not dilute or mix Pulmozyme with other drugs in the nebulizer
    • Mixing of Pulmozyme with other drugs could lead to adverse physicochemical and/or functional changes in Pulmozyme or the admixed compound
  • Follow the manufacturer's instructions on the use and maintenance of the equipment, including cleaning and disinfection procedures

Have your patients download patient-friendly, step-by-step instructions on administering Pulmozyme.

Cystic Fibrosis Foundation Guidelines recommend Pulmozyme in CF patients* 24

For individuals with CF, 6 years of age and older, with asymptomatic or mild lung disease, the CF Foundation recommends the chronic use of dornase alfa to improve lung function and reduce exacerbations. 

Mild disease (FEV1 ≥ 70% of predicted)
Certainty of net benefit: High
Estimate of net benefit: Moderate
Grade of recommendation: B

For individuals with CF, 6 years of age and older, with moderate to severe lung disease, the CF Foundation strongly recommends the chronic use of dornase alfa to improve lung function and reduce exacerbations. 

Moderate to severe disease
(FEV1 ≤ 69% of predicted)
Certainty of net benefit High
Estimate of net benefit: Substantial
Grade of recommendation: A

*Adapted from Mogayzel. Cystic Fibrosis Pulmonary Guidelines. Am J Respir Crit Care Med. 2013.  

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.

You may report side effects to the FDA at 1-800-FDA-1088 or www.fda.gov/medwatch. You may also report side effects to Genentech at 1-888-835-2555.

For further information, please see the Pulmozyme full Prescribing Information.

Indication

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.

Pediatric Use

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%).