Instructions for how to administer Pulmozyme with a Jet Nebulizer and Compressor18

Jet Nebulizer image

There are 2 ways to administer Pulmozyme, with a Jet Nebulizer and Compressor or with a portable nebulizer like the  eRapid™ Nebulizer System. For instructions using the portable eRapid™ Nebulizer System, go here

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Instructions for Use with Jet Nebulizers and Compressors18

A nebulizer and a compressor are used together to give a dose of Pulmozyme. 
A nebulizer changes the Pulmozyme liquid medicine into a fine mist inhaled by breathing through a mouthpiece. A compressor gives the nebulizer power and makes the nebulizer work.
Pulmozyme should only be used with the approved nebulizers and compressors or with the eRapid® Nebulizer System.

Any other inhaled medicines should not be used in the nebulizer at the same time. Keep all other inhaled medication systems completely separate from Pulmozyme. 

A mask should not be used. The mouthpiece provided with each nebulizer kit should be used. 
If a child cannot breathe in or breathe out by mouth, you may use the PARI BABY
reusable nebulizer, but should first discuss it with a doctor. The PARI BABY nebulizer is the same as the PARI LC Plus Jet system, except the mouthpiece is replaced by a tight-fitting face mask connected to an elbow piece.


Steps to administer Pulmozyme using approved jet nebulizer systems

Supplies you will need to give a dose of Pulmozyme:

Ampule of Pulmozyme® (dornase alfa)

1 ampule of Pulmozyme

Compressor

Compressor

Nebulizer Cup and Cap

Nebulizer cup and screw-on or snap-on cap

Plastic T

Plastic T connector (not needed for Sidestream nebulizer or PARI BABY™)

Tube

Flexible aerosol tube (not needed for Sidestream nebulizer or PARI BABY™)

Mouthpiece

Clean mouthpiece or PARI BABY™ facemask

Reservoir Tube

Long connecting tube

Nose Clip

Nose clip (optional, not needed for PARI BABY™)

Figure A

Remember to communicate these important instructions to patients/caregivers:

Step 1. Clean a flat table surface and wash your hands

  • Clean a flat table surface
  • Wash your hands well with soap and water before using the Pulmozyme ampule and nebulizer. This helps prevent infection (See Figure B)
Wash hands
Figure B

Step 2. Gather the nebulizer and test the compressor

  • Place the nebulizer parts on a clean, flat table surface within reach 
  • Test the compressor by turning it on and putting your finger in front of the "air out" or "air" port to feel air flowing. Turn off the compressor (See Figure C)
  • For the MOBILAIRE™ compressor only, turn the pressure control knob fully to the right for the highest pressure output
On/off button
Figure C

Step 3. Gather the Pulmozyme ampule and check the expiration date

  • Remove 1 foil pouch of Pulmozyme from the refrigerator. Open the foil pouch and remove 1 ampule of Pulmozyme. Put the remaining ampules back in the foil pouch and return them to the refrigerator
  • Check the expiration date printed on the ampule (See Figure D). Do not use the Pulmozyme ampule if the expiration date has passed
Ampule
Figure D

Step 4. Check the Pulmozyme ampule

  • Check the ampule for leaks by turning it upside down and gently squeezing (See Figure E). Do not use the ampule if it is leaking. Throw it away and get a new one
  • Check the Pulmozyme liquid in the ampule and make sure it is clear and free of particles
  • Do not use Pulmozyme if the liquid is cloudy or discolored. Take the Pulmozyme back to the pharmacy, hospital, or clinic that gave you the medicine
Ampule
Figure E

Step 5. Attach the tube to the compressor

  • Attach the long connecting tube to the "air out" or "air" port on the compressor (See Figure F)
Tubing
Figure F

Step 6. Attach the mouthpiece.

  • Skip to Step 7 if you use the Sidestream nebulizer or the PARI BABY™ nebulizer
  • Push the mouthpiece into the wider end of the plastic T. Attach the flex tube to the other end of the T (See Figure G)
Mouthpiece
Figure G

Step 7. Remove the cap from the cup

  • Unscrew or unsnap the cap from the nebulizer cup (See Figure H) 
  • Put the nebulizer cup on the table face up and place the cap upside down on a clean surface (See Figure I)

Nebulizer cup
Figure H
Nebulizer cup face up
Figure I

Step 8. Open the Pulmozyme ampule

  • Hold the tab at the bottom of the Pulmozyme ampule firmly. Twist off the top. Do not squeeze the body of the ampule (See Figure J)
Open ampule
Figure J

Step 9. Pour the full Pulmozyme dose into the nebulizer

  • Turn the ampule upside down and squeeze gently to empty the medicine into the nebulizer cup. Keep squeezing until the ampule is empty. It is very important you squeeze all of the medicine out of the ampule (See Figure K)
  • Screw or snap the cap onto the nebulizer cup (See Figure L)

Pour ampule into cup
Figure K
Green light and mist
Figure L

Step 10. Connect the plastic T

 

  • Connect the plastic T to the nebulizer cap (See Figure M)
  • If you are using the Sidestream nebulizer, attach the mouthpiece to the top of the nebulizer (See Figure N)
  • If you are using the PARI BABY™ nebulizer, connect the elbow piece and mask to the nebulizer outlet (See Figure O)

 

Cap
Figure M
Mouthpiece
Figure N
Elbow and mask
Figure O

Step 11. Attach the tube to the cup

  • Connect the open end of the long tube to the port on the bottom of the nebulizer cup by pushing up firmly (See Figure P)

Connect tube to cup
Figure P

Step 12. Turn on the compressor

  • Turn on the compressor and check to see that mist is coming out of the nebulizer (See Figure Q)
  • If you are using the MOBILAIRETM compressor, turn the compressor control knob fully to the right, then turn on the compressor. The pressure gauge should move between 35 psi and 45 psi, the highest pressure output (See Figure R)
Mist
Figure Q
Gauge and knob
Figure R

Taking a dose of Pulmozyme with a nebulizer:

Step 13. Breathe through the mouthpiece.

  • Skip to Step 14 if you are using the PARI BABY to give Pulmozyme to your child
  • Place the mouthpiece between your teeth and on top of your tongue (See Figure S) 
  • Breathe slowly in and out through your mouth. Do not block the airflow with your tongue
  • Do not breathe through your nose. If you have problems breathing only through your mouth, use a nose clip (See Figure T)
  • Do not be concerned if liquid droplets form in the long connecting tube during treatment. When the nebulizer begins spitting, gently tap the nebulizer cup and continue breathing until the nebulizer cup is empty or stops making mist (See Figure U)
  • If you need to stop treatment before you are finished, or you begin coughing, turn off the compressor and do not spill any of the medicine
  • To start treatment again, turn on the compressor and continue breathing slowly in and out through your mouth
  • The complete treatment usually takes from 10 to 15 minutes for most nebulizers and compressors
  • If you are using the Sidestream nebulizer with the MOBILAIRE™ or Porta-Neb compressors, treatment usually takes from 2 to 6 minutes
  • It is important to inhale the full dose of Pulmozyme. If you find a leak or feel any moisture coming from the nebulizer during treatment, turn off the compressor and check to make sure the nebulizer cap is sealed correctly before continuing (See Figure V)
Mouthpiece
Figure S
Nose clip on
Figure T
Continue breathing
Figure U
Sealed tight
Figure V

If you are using the PARI BABY™ nebulizer to give Pulmozyme to your child, follow the instructions below in Step 14. If not go to Step 15.

Step 14. Breathing through the facemask 

During the treatment, your child may sit, lie down, or stand.

  • Place the facemask gently but firmly over your child’s nose and mouth (See Figure W) 
  • Make sure there are no air gaps between the mask and your child's face. This will help make sure that your child will get the full dose of Pulmozyme
  • It is important that you try to keep the body of the nebulizer upright during the entire treatment (See Figure W). The elbow piece will allow you to move the mask for a good fit while keeping the nebulizer body upright
  • When the nebulizer begins "spitting," gently tap the nebulizer cup and continue treatment until the nebulizer is empty or stops making a mist (See Figure X)
  • If you need to stop the treatment or your child begins to cough during treatment, turn the compressor off. Do not spill any Pulmozyme
  • If you have not removed the mask and you want to begin the treatment again, turn on the compressor
  • If you have removed the mask, repeat the steps above to replace the mask on your child’s face and restart the compressor 
  • The complete treatment usually takes from 10 to 15 minutes

It is important that your child inhale the full dose of Pulmozyme. If you find a leak or feel moisture coming from the nebulizer during the treatment, turn off the compressor and make sure the nebulizer cap is sealed correctly before starting the compressor again (See Figure Y).

Place facemask on
Figure W
Tap the Nebulizer
Figure X
Seal Nebulizer cap
Figure Y

After your treatment with Pulmozyme:

Step 15. Prepare the nebulizer for cleaning and storage.

  • Turn off the compressor and take apart the nebulizer system. Set aside the flex tube and the long connecting tube
    Note: The Sidestream nebulizer does not use a flex tube
  • Throw away the Pulmozyme ampule in your household trash
  • Follow the manufacturer's recommendations for care of your nebulizer and compressor

How should I store Pulmozyme?

  • Store Pulmozyme in its foil pouch in the refrigerator between 36°F to 46°F (2°C to 8°C) until you are ready to use it
  • When traveling, Pulmozyme should be kept cold
  • Do not freeze Pulmozyme
  • Protect Pulmozyme from excessive heat and strong light
  • Do not use Pulmozyme if it has been left at room temperature for a total time of 24 hours or if it becomes cloudy or discolored
  • Do not use Pulmozyme past the expiration date printed on the ampule


Indication and Usage

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.

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.

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%).

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.

Please see full Pulmozyme Prescribing Information for additional Important Safety Information.

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    Non-US Residents visit: Pulmozyme.global