Version May 2024
Cystic fibrosis: Inhalation: 2.5 mg once daily through selected jet nebulizers in conjunction with an air compressor system (Durable Sidestream, Hudson T Up-draft II, Marquest Acorn II, PARIBABY, or PARI LC Plus) or eRapid Nebulizer System. Some patients may benefit from twice daily administration.
Note: Patients unable to inhale or exhale orally throughout the entire treatment period may use Pari-Baby nebulizer.
Parapneumonic effusions, complicated and empyema (adjunctive agent) (off-label use):
Note : Consider in combination with thrombolytic in patients who do not sufficiently respond to conventional therapy (eg, chest tube drainage, antibiotics) and/or who are not surgical candidates (Ref). Optimal regimen and doses have not been identified; refer to institutional protocols.
Intrapleural: 5 mg once or twice daily for 3 days; used sequentially or concurrently with intrapleural alteplase. For sequential dosing, administer alteplase first, then administer dornase alfa and dwell for ~1 hour and drain for ~1 hour (Ref). For concurrent dosing, administer dornase alfa and alteplase via 2 separate syringes, followed by a 10 to 60 mL saline flush; dwell for ~1 to 2 hours then drain (Ref).
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer's labeling.
Refer to adult dosing.
(For additional information see "Dornase alfa: Pediatric drug information")
Cystic fibrosis, acute exacerbations and chronic use:
Infants (limited data available in <3 months of age), Children, and Adolescents: Inhalation: Nebulization: 2.5 mg once daily through eRapid nebulizer or selected jet nebulizers in conjunction with an approved air compressor system (Ref). Some older patients (generally, those >21 years of age) or patients with baseline forced vital capacity >85% may require twice-daily dosing (Ref).
Rhinosinusitis, chronic; secondary to cystic fibrosis: Limited data available: Children ≥5 years and Adolescents: Inhalation: Intranasal: 2.5 mg once daily nebulized intranasal with the Pari Sinus; dosing based on double-blind, placebo-controlled cross-over trial in 23 subjects (age range: 5 to 67 years) which showed improvement of primary nasal symptoms compared to placebo (isotonic saline); treatments were well tolerated; nasal bleeding was reported in 2 patients (Ref).
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer's labeling.
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified. Adverse events were similar in children using the PARI BABY nebulizer (facemask as opposed to mouthpiece) with the addition of cough.
>10%:
Cardiovascular: Chest pain (18% to 25%)
Central nervous system: Voice disorder (12% to 18%)
Dermatologic: Skin rash (3% to 12%)
Respiratory: Cough (PARI-BABY nebulizer facemask: children 3 months to <5 years: 45%; children 5 to ≤10 years: 30%), pharyngitis (32% to 40%), rhinitis (30%; in patients with FVC: <40%), decrease in forced vital capacity (≥10% decrease of predicted: 22%; in patients with FVC: <40%), dyspnea (17%; in patients with FVC: <40%)
Miscellaneous: Fever (32% in patients with FVC <40%)
1% to 10%:
Gastrointestinal: Dyspepsia (≤3%)
Immunologic: Antibody development (to dornase alfa: 2% to 4%)
Ophthalmic: Conjunctivitis (1% to 5%)
Respiratory: Laryngitis (3% to 4%)
<1%, postmarketing and/or case reports: Headache, urticaria
Hypersensitivity to dornase alfa, Chinese hamster ovary cell products, or any component of the formulation
Disease-related concerns:
• Decreased pulmonary function: In patients with pulmonary function <40% of normal, dornase alfa does not significantly reduce the risk of respiratory infections that require parenteral antibiotics.
Some adverse effects were reported more frequently in infants and children <5 years of age than older children (5 to 10 years of age) including cough (45% vs 30%), rhinitis (35% vs 27%), and rash (6% vs 0%).
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution, Inhalation:
Pulmozyme: 2.5 mg/2.5 mL (2.5 mL)
No
Solution (Pulmozyme Inhalation)
2.5 mg/2.5 mL (per mL): $62.34
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution, Inhalation:
Pulmozyme: 2.5 mg/2.5 mL (2.5 mL)
Nebulization: Administer via the eRapid Nebulizer System or via a jet nebulizer (Durable Sidestream, Hudson T Up-draft II, Marquest Acorn II, PARIBABY, or PARI LC Plus) connected to an approved air compressor (Pulmo-Aide, PariProneb, Mobilaire or PortaNeb) with air flow of ~3.5 to 9 L/minute at ~20 to 45 psi pressure and equipped with a face mask or mouthpiece. Prior to use, squeeze each ampul to check for leaks. Once ampul is opened, the entire contents must be used or discarded. Do not dilute or mix with any other drugs in the nebulizer, this may inactivate the drug. When administered with the eRapid Nebulizer System, replace handset after 90 uses to ensure delivery of appropriate dose. Follow the manufacturer's instructions on use and maintenance of the equipment.
Intrapleural: Parapneumonic effusions, complicated and empyema (off-label use):
Sequential administration: For sequential administration of alteplase and dornase alfa, dilute each dose in 30 to 50 mL NS or sterile waterr; administer alteplase first and let dwell, then drain; after alteplase drain, administer dornase alfa, followed by a saline flush (eg, 10 to 25 mL); dwell for ~1 hour and drain for ~1 hour. Stability of dornase alfa diluted in NS or sterile water has not been formally evaluated; use immediately after preparation. Instill dose into chest tube and clamp drain. After ~1 hour dwell time, release clamp and connect chest tube to continuous suction to drain (Ref).
Concurrent administration: For concurrent administration of alteplase and dornase alfa, using separate syringes, dilute dornase alfa dose in 30 to 50 mL NS or sterile water. Stability of dornase alfa diluted in NS or sterile water has not been formally evaluated; use immediately after preparation. Instill alteplase and dornase alfa into chest tube, one immediately after the other, followed by a 10 to 60 mL NS flush. Clamp drain for ~1 to 2 hours, then drain (Ref).
Inhalation:
Nebulization: Should not be diluted or mixed with any other drugs in the nebulizer per the manufacturer's labeling; this may inactivate the drug. Dilution in NS (1 to 5 mL) has been reported in neonates (Ref). Administer through eRapid Nebulizer system or select jet nebulizers (eg, Hudson T Up-Draft II, Marquest Acorn II, Pari LC Plus, Pari Baby, Durable Sidestream) in conjunction with an air compressor system (eg, Pulmo-Aide Pari-Proneb, Mobilaire, Porta-Neb).
Intranasal: Nebulize for 4 minutes per nostril using Pari Sinus compressor and Pari LC Sprint Star (Ref).
Cystic fibrosis: Management of cystic fibrosis patients, in conjunction with standard therapies, to improve pulmonary function; reduce the risk of respiratory tract infections requiring parenteral antibiotics in patients with a forced vital capacity (FVC) ≥40% of predicted.
Parapneumonic effusions, complicated and empyema
None known.
There are no known significant interactions.
Adverse events have not been observed in animal reproduction studies.
Measurable amounts would not be expected in breast milk following inhalation; however, it is not known if dornase alfa is present in breast milk. According to the manufacturer, the decision to breastfeed during therapy should take into account the risk of exposure to the infant and the benefits of treatment to the mother.
The hallmark of cystic fibrosis lung disease is the presence of abundant, purulent airway secretions composed primarily of highly polymerized DNA. The principal source of this DNA is the nuclei of degenerating neutrophils, which is present in large concentrations in infected lung secretions. The presence of this DNA produces a viscous mucous that may contribute to the decreased mucociliary transport and persistent infections that are commonly seen in this population. Dornase alfa is a deoxyribonuclease (DNA) enzyme produced by recombinant gene technology. Dornase selectively cleaves DNA, thus reducing mucous viscosity and as a result, airflow in the lung is improved and the risk of bacterial infection may be decreased.
Onset of action: Nebulization: Enzyme levels are measured in sputum in ~15 minutes and decline rapidly thereafter
Duration: Sputum concentrations decline within 2 hours of inhalation
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