Everyone has mucous lining their airways. Normal mucous is thin and is essential to a healthy airway. In CF airways, this mucous is thick and sticky. The presence of bacteria calls for white blood cells to fight infection but eventually the white blood cells die. So along with different bacteria and fungi in the airways, CFers also have the waste products of dead white blood cells (DNA). This just thickens the mucous even more.
Nebulized Medications used to aid in Airway Clearance
Bronchodilators such as Albuterol or Xopenex help open the airways that move air to or from the lungs.
Mucolytics loosen the mucous. Hypertonic Saline hydrates the airways and mobilizes mucous. Pulmozyme breaks down the leftover DNA from dead white blood cells. This helps CFers cough out the thick secretions.
The difference between Hypertonic Saline and Pulmozyme and how they can work together.
Before the CF Conference I was under the impression that Hypertonic Saline and Pulmozyme had the same basic function, to thin the mucous, but learned that both function quite differently. Often used in conjunction, the two drugs have amazing results and have proven to raise PFT's (pulmonary function test) and reduce the occurrence of infection.
Here are two photos I borrowed from Pulmozyme's website for Health Care Professionals, edited to make sense to me and you of course.
Airway Loosening vs. Airway CLEARance
If you have an CF infant or toddler like me, you are probably doing your clinic's recommended nebulized medications and airway clearance techniques (ACTs) but your child may or may not be expectorating or coughing out any mucous. Yes, bronchodilators used in conjunction with mucolytics and different ACTs (the Vest in our case) do an amazing job at loosening the mucous but the main goal is to clear the mucous out of the lungs.
Ways to get rid of the mucous
The main goal of Airways Clearance is to CLEAR the lungs of the thick, sticky, bacteria ridden mucous. In an ideal world, a CFer would perform all the recommended treatments and then cough the mucous up and out of the lungs. Sometimes I hear Nathan cough up mucous and then swallow really hard. It would be better for him to cough the mucous out than to swallow the empty calories creating the feeling of fullness and possibly introducing unnecessary bacteria to another system of the body. But nevertheless it is better to have to mucous out of the lungs!
So we've been working on ways to get Nathan coughing. My recommendation is to get your baby or toddler laughing. Do whatever you have to do to get the air moving...tickling works as does making a fool of yourself, acting like a gorilla or Grover, making strange noises, etc.
I've also noticed that kids are sponges and copy everything adults do so why not cough yourself and hope your little copy cat does the same, it works for us. Just last night we asked Nathan to cough, shockingly he complied. Of course it was followed by the "good boy" reinforcement letting him know that this is an action we approve of.
Ways to introduce your baby/toddler to Airway CLEARance:
1. BLOWING - Teach your child to blow bubbles (not eat them), windmills, into straws. All are great introductions to huffing (a controlled breathing technique/gentle cough which ultimately releases the trapped air from the smaller airways).
2. BOUNCING/JUMPING - Trampolines are not only a great way to get the air moving and cause coughing but good for getting that albuterol related energy out. Bounce your baby on your knee or bounce with your baby/toddler on an exercise ball.
Websites I recommend visiting The Cystic Fibrosis Learning Center set up the Continuum of Care Conference which targeted healthcare professionals from CF care centers and family members/caregivers of patients with CF. If it were not for the generous donations that made it possible for the ladder audience to attend free of charge my husband and I would not have been able to attend. So a huge thank you to the donors!
Pulmozyme's Cystic Fibrosis Community is "a place for people of all ages to connect and learn about life with cystic fibrosis. There are [age appropriate] stories, games, links, tips, and much more."
There will be many more websites to come, these are only a few for now. I don't want to overwhelm anyone.