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Medications for CF
In addition to ACT, people with CF also take many inhaled medications to keep the lungs as healthy as possible, open the airways, thin the mucus, and fight infection. It is critical for the person with CF to use a technique or a combination of techniques consistently.
The CF pulmonary guidelines for the administration of inhaled medications include the following:2
- Bronchodilators: These help to open the airways and improve the effectiveness of ACT. Additionally, other inhaled medications can cause bronchospasm, and taking the bronchodilator first helps to protect against this reaction.
- Hypertonic saline: This helps to draw more fluid into the airways helping to thin the mucus, making it easier to cough out. For this reason, hypertonic saline should be taken prior to ACT.
- Recombinant human rhDNase (DNase): Also known as Pulmozyme® (Genentech), this medication thins mucus by breaking apart the DNA left over from white blood cells that fight infection. Many people with CF do their ACT while inhaling their DNase, and others do it afterward.
- Antibiotics: These medications are to be done last so they can have improved disposition throughout the lungs because of the previous medications and ACT that helped to get as much mucus out of the lungs as possible. Inhaled antibiotics help to reduce the number of bacteria in the lungs, while the inhaled corticosteroids help reduce underlying inflammation.
- Corticosteroids: People with CF who also have asthma or allergic bronchopulmonary aspergillosis should take these medicines last to promote medication disposition.
Using the right nebulizer
The nebulization of medication differs with equipment used. Various compressors and nebulization systems provide different droplet sizes that may affect the amount of medication that is actually delivered to the airways. In general, the lower the pressure of the compressor, the larger the droplet size of the medication. Since droplets greater than 5 microns are not inhaled deeply into the lungs, it is important to make sure the home nebulizer is working properly. It also means portable compressors, which may make treatments more convenient, are not as effective in delivering the medication.
Each drug should be given separately in order to be most effective. Inhaled antibiotics such as tobramycin solution for inhalation (TOBI®, Novartis) or inhaled sterile colistimethate sodium (Colymycin, USP) are dose-dependent and should be given using a breath-enhanced nebulizer that wastes less of the medication during exhalation and in turn provides a greater dose. Bronchodilators and DNase are fine to be given using a sidestream device but also may be given via a breath-enhanced nebulizer.
Finally, cleaning and disinfecting the nebulizer is important. The therapist must stress that family members with CF should not share equipment such as nebulizer cups in order to avoid sharing germs as well.
While it is important to follow the manufacturer's recommendations for cleaning and disinfecting, it is just asimportant to help patients choose equipment that can be disinfected properly. In general, nebulizer parts may be cleaned with hot water and a mild liquid soap. After cleaning, disinfecting may be done using a variety of methods including a bleach solution, boiling water, or hydrogen peroxide. Vinegar is not adequate for disinfecting equipment. 3
What to teach your patient
An RT is the primary educator for many of the ACT and nebulized therapies. Patients and their families should learn proper techniques for various airway clearance therapies and have the opportunity for return demonstration. Every patient encounter provides time to review the basics, assess effectiveness and understanding, and help problem-solve issues.
The RT who takes a few minutes to ask questions can uncover many issues such as improper order of medications, which nebulizer is being used for each drug, equipment that is ineffective, non-adherence, or poor technique.
In addition, the RT can teach patients and family members about other issues such as good hand washing techniques, the importance of recommended vaccinations and annual flu shots, exercise, and even tips on how to fit all the therapies into a daily schedule.
View a list of references.
Jonathan M. Rosen, MD, is associate dean for medicl education and associate professor of medicine, Albany Medical College, New York. Leslie Hazle, MS, RN, CPN, joined the CF Foundation in 2001 as the director of patient resources. Penny Morgan Overgaard, RN, BSN, FAHCEP, is a nurse in the Trach & Airway Program at Phoenix Children's Hospital. Melissa Chin, BA, joined the CF Foundation in 2008 as the clinical programs project coordinator.
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