Preparing for CF treatment with Pulmozyme

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Video for patients about taking Pulmozyme

See step-by-step instructions on how to take Pulmozyme


Instructions

Before taking or administering Pulmozyme, it is important that you receive training from your CF Care Team.

Clean

  1. Begin by cleaning a flat table surface with isopropyl alcohol, also known as rubbing alcohol.
  2. Always wash your hands thoroughly with soap and water before preparing the medication. This will avoid the risk of germs being introduced into the system.
  3. Place the nebulizer system onto the clean surface. Do not dilute or mix Pulmozyme with other drugs in the nebulizer. The system should be clean and ready for use.

 

Test

  1. Test the compressor by turning it on, and feel the air output by putting your finger in front of the air output port. Turn the compressor off.
  2. Remove one ampule of Pulmozyme from its package. Look at the expiration date. Do not use if it is past the expiration date. Look at the contents of the solution to be sure it is clear. If the solution is cloudy or discolored, do not use it. Turn the ampule upside down and squeeze gently to ensure no leak is present.

 

Setup

  1. Attach the tubing to the air output nozzle of the compressor. Attach the other end to the bottom of the nebulizer.
  2. Twist the top off the ampule of Pulmozyme. Empty the contents into the medicine cup of the nebulizer.

 

Administer

  1. Now, attach the mouthpiece or facemask to the nebulizer. Check to ensure the tubing is properly connected to the bottom of the nebulizer. Turn the compressor on. You should see mist being generated.
  2. The mouthpiece should be placed between teeth and on top of the tongue. The tongue should not block the flow of medicine. Take slow, deep breaths, making sure to avoid nose breathing. 10. Toward the end of the treatment, when the nebulizer begins spitting, gently tap the medicine cup to ensure all of the medicine has been used. When the mist subsides, your treatment is complete
  3. Toward the end of the treatment, when the nebulizer begins spitting, gently tap the medicine cup to ensure all of the medicine has been used. When the mist subsides, your treatment is complete.

How to store Pulmozyme

  • Pulmozyme should be refrigerated (2°C to 8°C/36°F to 46°F) in the protective foil to protect from light
  • Do not use beyond the expiration date stamped on the ampule
  • Store unused ampules in their protective foil pouch in the refrigerator
  • Refrigerate Pulmozyme when traveling and do not expose to room temperatures for a total time of 24 hours

What you’ll need to take Pulmozyme

Pulmozyme Dose

Ampule of Pulmozyme® (dornase alfa)

One ampule of Pulmozyme

Pulmozyme is supplied in single-use, disposable ampules. It should be refrigerated and protected from strong UV light.

Equipment

Nebulizer

One Nebulizer

One nebulizer approved for use with Pulmozyme.

Nebulizer tubing

Nebulizer Tubing

Nebulizer tubing and other components that come with the nebulizer.

Mouthpiece and compressor

Mouthpiece and compressor

A mouthpiece (or a face mask for children younger than 5 years of age) and a compressor compatible with your nebulizer.


Establishing regular routines for CF treatments early can pay off later

  • Set small goals—like taking Pulmozyme daily—to help you stay on track
  • Continue to be your own health advocate. Ask your CF Care Team any questions you may have about your medications and report any concerns or any side effects you may be experiencing
  • Take advantage of your hobbies. Include activities like watching a movie or reading a story during your treatment routine to help make it a little easier, so it feels less like a chore
  • Start early. Habits learned in youth can carry through into adulthood. It’s easier to wake up early for a nebulizer treatment if you’ve been doing it for years, and understanding CF at a young age helps reinforce the importance of taking CF seriously

Considerations for Children

Special considerations for Parents & Caregivers

It’s important to help your child develop healthy ways of dealing with any problems they may have. As children with CF get older, they seek more independence. Establishing clear communication with consistent treatment routines can smooth the transition from childhood to adulthood. Here are some ways you can help.

Listen to your child.

They are experiencing a lot of different emotions and may need someone to talk to. Let them know you’re there to help them work through any feelings they may be having.

Create a safe place.

Older children may begin to feel pressured to fit in with their friends at school. You can help them by making your home as comfortable as possible. Encourage the rest of your family, including siblings, to be supportive as well.

Maintain trust and clear communication.

As teenagers are more independent than preteens, they may resist your efforts to manage every aspect of their care. By stressing the importance of honesty and transparency, you can allow them to handle more and more of their treatment while avoiding “helicopter parenting”.

Emphasize the benefits of sticking to routines, and not the negative consequences.

More consistent treatment means more quality time with friends and more time for the activities your child loves. These positive outcomes can motivate your child to create a treatment routine.

Make sure to have some fun.

You and your child are dealing with a busy schedule, so it’s important that you both have time for fun activities. Set aside some time each week to go to the park or cook a special dinner together.

When it comes to school.

Give your son or daughter a letter outlining their CF treatment that they can share with their teachers and school nurse. This may help your child explain the basic facts of their condition and treatment process. You can write the letter yourself, with advice from a doctor.

Your child likely qualifies for an Individual Education Plan (IEP) under the Individuals with Disabilities Education Act (IDEA). 

Your CF doctor can work with the school administration to establish your child’s needs, and lay the foundation for accommodations, such as excused absences and tutors for when your child is too sick to attend school.

Stress the importance of daily routines, even if they require waking up early for treatment.

Calendar

The Cystic Fibrosis Foundation Guidelines recommend regular visits to your child's primary doctor and cystic fibrosis care center so they can help you manage your child's cystic fibrosis.

The safety and effectiveness of Pulmozyme have been established in 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 cystic fibrosis patients who may experience potential benefit in pulmonary function, or who may be at risk of respiratory tract infection.

Helpful resources

Get more tips and helpful resources

Indication & Usage

Pulmozyme (dornase alfa) is indicated for daily administration along 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 injectable antibiotics.

Important Safety Information

Pulmozyme should not be used in patients who are allergic to any of its ingredients.

Patients may experience the following when using Pulmozyme: change in or loss of their voice, discomfort in the throat, rash, chest pain, red watery eyes, runny nose, lowering of lung function, fever, indigestion, and shortness of breath. There have been no reports of severe allergic reactions caused by the administration of Pulmozyme. Mild to moderate hives and mild skin rash have been observed and have been short-lived.

Pediatric Use

The safety and effectiveness of Pulmozyme have been established in patients 5 years of age and older. While clinical trial data are limited in patients younger than 5 years of age, the use of Pulmozyme should be considered for pediatric CF patients who may experience potential benefit in lung function or who may be at risk of respiratory tract infection.

The safety of Pulmozyme given by daily inhalation for 2 weeks has been studied using 98 CF patients with 65 of them aged 3 months to <5 years (younger group) and 33 aged 5 years to <10 years (older group). The PARI BABY™ reusable nebulizer (which uses a face mask instead of a mouthpiece) was used in patients who were unable to show that they could breathe in or out using their mouth throughout the entire treatment period. Overall, the kind of side effects observed in children was similar to those seen in larger trials in older patients.

You are encouraged to report side effects to Genentech and the FDA. 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. If you have questions, please discuss them with your CF Care Team.