Understanding Cystic Fibrosis: Causes, Symptoms, and Treatment

Cystic Fibrosis patient icon

Cystic fibrosis (CF), unlike chronic obstructive pulmonary disease (COPD) or bronchiectasis, is a genetic condition that affects more than 30,000 people in the United States.[1] According to the Cystic Fibrosis Foundation, “People with CF have inherited two copies of the defective CF gene—one copy from each parent. Both parents must have at least one copy of the defective gene.”[2]

Though CF is different from other chronic lung conditions since it affects other major organs, as well as the digestive system[3], it does share similarities with COPD and bronchiectasis. For example, CF is a progressive disease that may cause an individual to experience frequent flare ups (i.e., worsening of symptoms) that permanently damage the lungs and make breathing more difficult.

How Does Cystic Fibrosis Affect the Body?

In a previous post, we discuss how our bodies use mucus to trap bacteria, germs, and airborne allergens[4]. Once these particles are blocked by mucus, cilia—which are tiny hair-like structures in your lungs—get to work to help clear mucus from the airways, preventing build up and the spread of bacteria.

When an individual is living with CF, however, the defective gene causes the body to produce thick, sticky mucus that the cilia is unable to remove: “Instead of acting as lubricants, the secretions plug up tubes, ducts and passageways, especially in the lungs and pancreas.”[5]

The more mucus that builds up, the more the body is exposed to bacteria, which leads to infection.[6] And with each infection, the lungs become more damaged and a person may experience a worsening of CF symptoms.

What Are Common Cystic Fibrosis Symptoms?

Young child wearing a SmarVest.Because CF is inherited, children can begin to show signs of this disease before they turn one, but as with most chronic respiratory diseases, it will depend on each individual’s unique circumstance.[7]

According to the Cystic Fibrosis Foundation, common symptoms of CF include:

  • Frequent coughing that may produce phlegm
  • Repeated lung infections
  • Wheezing
  • Shortness of breath
  • Obstructed airways
  • Nutritional deficiencies[8]

In some cases, a child may not develop CF symptoms until they are older.[9] Nevertheless, without effective treatment, CF can worsen over time and create serious complications, including damaged airways, which can cause a person to experience symptoms of bronchiectasis.[10]

Though there is no known cure for CF, there are treatment options available to help relieve symptoms and reduce flare ups that require hospitalization.

How to Clear the Airways

Airway Clearance Therapy (ACT) is an essential treatment.[11] Whether it’s performed manually or using a device, such as a respiratory vest, airway clearance helps loosen mucus from the lungs, so it is more easily coughed out—thereby preventing mucus build up.

cystic fibrosis SmartVest therapy, CF vest therapy

ACT is often prescribed with other treatment options, including inhaled bronchodilators to help relax the muscles and widen airways before performing ACT, antibiotics to fight infection, and certain medications that help thin mucus so it’s easier to loosen.[12]

Whether your child is living with a genetic condition like CF or has developed symptoms of bronchiectasis as a result of the disease, ACT provides a treatment option to prevent mucus build up and manage CF symptoms, so your child can breathe easier, spend more time with friends, and enjoy being a kid again.

“We haven’t had any respiratory infections, she hasn’t had to be on antibiotics, and I know that she is getting the best possible treatment and prevention possible. Thank you, SmartVest, for finding a way to treat patients big and small. I know that we will have more tomorrows because of what SmartVest does for Parker!” -Kristin

The Benefits of HFCWO Therapy

As noted, ACT may be performed manually—which involves a therapist or caregiver clapping on a patient’s chest wall in various positions—or with a respiratory vest, also known as high frequency chest wall oscillation (HFCWO) therapy—which delivers recurring pulses of air that gently squeeze and release the upper chest wall, loosening and thinning mucus from the lungs.

Though both are effective forms of ACT and each involves the practice of coughing and huffing—a breathing technique used to help clear the lungs of mucus[13]—there are some added benefits to using an HFCWO therapy device like the SmartVest Airway Clearance System rather than choosing manual chest physiotherapy (CP).

In a previous blog post on the benefits of using HFCWO therapy vs. manual CPT, we discussed three essential differentiators:

  • Independence – Instead of relying on a family caregiver or respiratory therapist to perform each therapy session, a person can perform HFCWO therapy on his/her own, from anywhere in the home.
  • Frequency – With SmartVest, each pulse is delivered with the same accuracy and frequency to the chest wall, meaning you never miss a beat with therapy. It also means that your family has more free time to be together, since all you need to do is sit, relax, and plug in your device.
  • Monitoring – Using SmartVest also means you have access to the SmartVest Connect From your smartphone to your desktop or tablet device, you can track therapy performance, view historical metrics, leave notes for your clinician, and log sessions daily. This ensures you and your care team stay on track with each therapy session!

“The manual airway clearance treatment I was giving him daily was always such a hard time for us both. SmartVest changed our lives. He can operate and turn on his own settings and machine and he loves it. I couldn’t believe how much it decreased his coughing and lung infections by 70%! SmartVest has even worked better than most antibiotics!” -Shelly

Today, we’d like to add three additional benefits to this list:

  • Effectiveness – SmartVest has proven studies that demonstrate its effectiveness in stabilizing lung function, reducing antibiotic usage, and decreasing hospital visits for bronchiectasis patients.
  • Ease of Use – When your child’s doctor prescribes SmartVest, you can expect immediate support and guidance from Electromed’s support team. We’ll help you set up a training session to learn how to use the vest, so you or your child can operate the device at home independently.
  • Lifetime Support – Every vest that gets delivered is backed with a promise from our team that you’ll receive lifetime support. We want to ensure your device works at optimal condition and that you feel like you have a strong advocate on your behalf. We offer articles, videos, chat support, phone support (give us a call at 800.462.1045), and other online resources to help you and your loved ones feel confident about your vest therapy and start experiencing symptom relief from its performance.

To learn more about the benefits of HFCWO therapy via the SmartVest Airway Clearance System, request an informational packet today.

Red button to click for request a patient packet.


Resources:

[1] Cystic Fibrosis Foundation. “About Cystic Fibrosis.” Retrieved from https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/#:~:text=More%20than%2030%2C000%20people%20are,is%20age%2018%20or%20older.

[2]  Cystic Fibrosis Foundation. “About Cystic Fibrosis.” Retrieved from https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/#:~:text=More%20than%2030%2C000%20people%20are,is%20age%2018%20or%20older.

[3]  Mayo Clinic. “Cystic Fibrosis: Symptoms & Causes.” Retrieved from https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700

[4] Everyday Health. “What is Mucus?” Retrieved from https://www.everydayhealth.com/mucus/

[5] Mayo Clinic. “Cystic Fibrosis: Symptoms & Causes.” Retrieved from https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700

[6] Cystic Fibrosis Foundation (YouTube Channel). “How Cystic Fibrosis Affects the Lungs. Retrieved from https://www.youtube.com/watch?v=omkp2VJTE3c&feature=youtu.be

[7] Medical News Today. “What to Know about Cystic Fibrosis in Children.” Retrieved from https://www.medicalnewstoday.com/articles/326138

[8] Cystic Fibrosis Foundation. “About Cystic Fibrosis.” Retrieved from https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/#:~:text=More%20than%2030%2C000%20people%20are,is%20age%2018%20or%20older.

[9] Medical News Today. “What to Know about Cystic Fibrosis in Children.” Retrieved from https://www.medicalnewstoday.com/articles/326138

[10] Mayo Clinic. “Cystic Fibrosis: Symptoms & Causes.” Retrieved from https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700

[11] The American Lung Association. “How Cystic Fibrosis is Treated.” Retrieved from https://www.lung.org/lung-health-diseases/lung-disease-lookup/cystic-fibrosis/treating-and-managing

[12] Cystic Fibrosis Foundation. “Airway Clearance Techniques (ACTs).” Retrieved from https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Airway-Clearance/Airway-Clearance-Techniques/

[13] Cystic Fibrosis Foundation. “Coughing and Huffing.” Retrieved from https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Airway-Clearance/Coughing-and-Huffing/

Keep Reading

Benefits of Exercise with COPD

Next Up

Prescribing Effective Airway Clearance Just Got Easier