Use the Right Therapies
There are currently three kinds of pulmonary therapies for cystic fibrosis (CF). One helps clear sputum out of your lungs; another helps control bacteria; and the third reduces inflammation.
Clearing Out Sputum
A powerful cough is your lungs’ way of getting rid of sputum. The less sputum, the less your airways get plugged, the fewer bacteria grow and the better you’ll breathe. But your regular cough often needs a boost, since CF sputum is thick and sticky and there’s usually a lot of it.
Some therapies “jiggle” the sputum, making it easier to cough out. These are called airway clearance techniques (ACTs) and include manual chest physiotherapy (CPT), when another person claps on your chest with their hands; vibrating devices and vests; devices that create a fluttering pressure as you blow through them; and good old-fashioned exercise. All of these ACTs work like hitting a ketchup bottle to get ketchup out.
Of course, you can also thin ketchup. Other clearing therapies thin the sputum so it’s easier to cough out. They include DNase (Pulmozyme®) and hypertonic saline.
These medications work by different means, so they cannot replace one another or be considered as alternatives. A person can use both therapies, and because they have to be inhaled using a nebulizer, both take a lot of time.
To minimize the bacteria in your lungs, you may be inhaling an antibiotic, such as TOBI® or Cayston®. Newer antibiotics are being developed. These antibiotics are usually inhaled with a nebulizer, so they may be time-consuming.
Some medications seem to lessen inflammation in the lungs. When the lungs are less inflamed, they make less sputum. Some children with CF have taken ibuprofen (such as Advil®) to reduce inflammation in the airways. But ibuprofen therapy has not been shown to help the lungs of adults with CF. If your pediatric CF caregiver gave you ibuprofen, talk with your adult CF caregiver about it and about any side effects you may be having.
Another medication that seems to reduce inflammation and boost lung function (FEV1) is the antibiotic azithromycin. Oddly, it is not the medication’s antibiotic effect but some other factor that appears to reduce inflammation.
Asthma can be a kissing cousin to CF. Some people seem to have both, so you may be or need to be on asthma inhalers and oral medication.
Occasionally you might need to take a course of corticosteroids when your asthma suddenly gets worse. The corticosteroids are usually taken by mouth and help reduce inflammation. Prednisone is the most commonly prescribed.
These corticosteroids are different than the steroids that some athletes have used illegally and they are generally safe in the short run. But if taken for more than a couple of weeks they may have serious side effects. Speak with your doctor about the possible side effects of corticosteroids.
What Else You Can Do