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Respiratory Care

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When an individual is diagnosed with Cystic Fibrosis, that person and their family becomes a part of their CF Care team, along with the doctors, nurses, dietitian, social worker and respiratory therapist.

As part of the CF Care team, a respiratory therapist will perform Pulmonary Function testing (PFT's or blowing test), which is how lung function is measured. During PFT testing and at yearly visits, the respiratory therapist will discuss lung function and airway clearance. The PFT results will be compared to the results from the visit before and sent to the doctor to review with you. Lung function is plotted on a graph at each visit so we can see how the results compare to not only the past visit, but to all of the visits in past years.

Lung function should be optimized at every age, and looking at lung function can help determine how healthy your lungs are. There are lots of ways to help keep the lungs clear of mucus. These treatments may be started at a very young age, maybe even as a newborn. Even though a person with CF may not show signs of breathing trouble, there may still be swelling and mucus in the lungs, so it is important to make sure the lungs stay clear.

Airway Clearance

During your visit to Riley, you will meet with a Respiratory Therapist that is part of the CF Team. We will help you understand what therapy you may use to help your lungs stay healthy. In the process, we talk a lot about Airway Clearance Techniques (ACT's). These techniques clear the airways of mucus that is causing or could cause infection. ACT's can be started at a very young age. If the diagnosis of cystic fibrosis is made after having newborn screening, we can start the chest percussion in the first few weeks of life.

We (respiratory therapists) work in the Pulmonary Function Lab every day and do the Pulmonary Function Testing (blowing test). This test will help your doctor and the CF Team track lung function.

Goals of airway clearance therapy (ACT's) are:

To prevent and reduce lung infection
To improve and maintain lung function

Cough:

Coughing is the most basic airway clearance technique. It clears mucus with high-speed airflow. Coughing should be done with each ACT. Huff coughing can be less tiresome and may work better.

Mucocilliary clearance:

Normally, mucus protects the airway from inhaled irritants. The mucus lays on the airway like a blanket of jelly. Hairs called cilia along the airway sweep the blanket of jelly with mucus in it upward so that it can be coughed out. In people with CF the mucus is thick and sticky and the cilia don't sweep like they should, making it difficult to get the mucus out.

Importance of airway clearance:

Loosens mucus so it can be cleared by coughing or huffing
Moves mucus from small to central airways to be coughed or huffed out
If air gets behind mucus and airflow is increased, mucus is pulled along
...the result is reduced lung infection and improved lung function.

Order of Therapy

The order of airway clearance therapy is important because of the effect that these therapies have on the lungs. Each medicine and therapy has a place in this order.