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Treatments

Respiratory Treatments

 

Understanding how these medications work and why you take them in specific order will help you appreciate the importance of timing.  Since CF care is individualized, some people may not be prescribed all the medications listed, in which case you just follow the order leaving out the medications that are not prescribed for you.

 

Step 1: Open the airways with a bronchodilator

(Example:  Albuterol, ProAir, Ventolin, Proventil)

A bronchodilator relaxes the smooth muscle of the airways, opening them up and allowing for better airflow and easier airway clearance.

 

Step 2:  Hydrate the airways with an osmotic agent

(Example:  Hypertonic Saline)

Inhalation of a 7% hypertonic saline solutions (HTS) aids in hydrating the thick mucus making it easier to move.  Since HTS can cause bronchospasm, it is recommended that a bronchodilator always be given first.

 

Step 3: Thin the mucus with a mucolytic

(Example:  Pulmozyme, Mucomyst)

Mucolytics thin the thick, sticky mucus in the lungs.  When the mucus is less think and sticky it is easier to mobilize and cough or huff out.

 

Step 4: Clear the mucus with an Airway Clearance Technique

(Example: CPT, Vest, acapella, huffing, PEP)

Once the airways are relaxed and more open, and the mucolytic has thinned the mucus, then it is time for an airway clearance technique (ACT).  An ACT will help to mobilize the mucus out of the smaller airways and into the larger ones where a cough or huff will remove it.  Commonly in CF, the mucus in the lungs have millions of bacteria, so getting out as much of it out as routine, it is OK to do steps 1,2,3 in that order while you are on the vest or receiving CPT

 

Step 5:  Kill the bacteria with an inhaled antibiotic

(Example:  Tobi, Tobi podhaler, Colistin, Cayston)

After your airway clearance and cough or huff maneuver has cleared your lungs of the loosened mucus, it is time for the inhaled antibiotic, which helps to kill the bacteria remaining in the lungs.

 

Step 6: Reduce the swelling in the airways with an anti-inflammatory agent

(Example:  Flovent, Advair)

CF airways can become inflamed from all the mucus and infection.  Anti-inflammatory medication will help reduce this swelling.  It is best to take this last, after all of the other medications are taken.  Always remember to rinse you mouth after inhaled steroids.

For more detail on the above medications, Click here

Click here for more detailed instructions from the CF Foundation on doing breathing treatments with your infant or toddler

Click here for instructions on how to clean your nebulizer

Please ask the CF team any questions you may have.