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
Before taking or administering Pulmozyme, it is important that you receive training from your CF Care Team.
Clean
- Begin by cleaning a flat table surface with isopropyl alcohol, also known as rubbing alcohol.
- 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.
- 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
- 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.
- 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
- Attach the tubing to the air output nozzle of the compressor. Attach the other end to the bottom of the nebulizer.
- Twist the top off the ampule of Pulmozyme. Empty the contents into the medicine cup of the nebulizer.
Administer
- 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.
- 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.
- 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
- Store Pulmozyme ampules at a refrigerated temperature between 2°C to 8°C (36°F to 46°F) in their protective foil pouch to protect from light and heat until you are ready to use them. When the protective foil pouch is opened, the unused ampules must be kept refrigerated in the protective foil pouch to protect from light and heat
- When traveling, Pulmozyme ampules should be kept cold in their protective foil pouch to protect from light and heat
- Protect Pulmozyme from excessive heat and strong light
- Do not use Pulmozyme if the ampules have been exposed to room temperature (22°C to 28°C/72°F to 82°F) for more than a total of 60 hours or if the solution has turned cloudy or discolored
- Do not use Pulmozyme past the expiration date printed on the ampule
What you’ll need to take Pulmozyme
Pulmozyme Dose
One ampule of Pulmozyme
Pulmozyme is supplied in single-use, disposable ampules. It should be refrigerated and protected from strong UV light.
Equipment
One Nebulizer
One nebulizer approved for use with Pulmozyme.
Nebulizer Tubing
Nebulizer tubing and other components that come with the nebulizer.
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
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.
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.