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Understanding cystic fibrosis.

Understanding your cystic fibrosis (CF) can help you become a more active participant in your health care, which can help you lead a fuller, healthier life. The following is an overview of what CF is and how it affects your body.

The basics.

CF is a genetic, rare disease that affects about 30,000 people in the United States1. It typically affects the lungs, but can also affect other organs, such as the pancreas, liver and digestive system.

In a person without CF, a protein called the cystic fibrosis transmembrane conductance regulator (CFTR) allows the movement of salt and chloride through the membranes of the lungs, pancreas and other organs. People with CF have a change (mutation) in the gene that makes CFTR. As a result, chloride becomes trapped in cells. Without proper movement of chloride, water cannot hydrate the cell surface. This causes a build-up of thick, sticky mucus in the lungs and other organs.

In the lungs, the mucus clogs the airways, causing infections and swelling (inflammation). In the digestive tract and pancreas, the mucus prevents enzymes from aiding in the digestion of food. CF can also cause a medical condition called cystic fibrosis-related diabetes (CFRD). This happens when the pancreas doesn’t make enough insulin to help control blood sugar, or when insulin doesn’t work the right way.


Symptoms for CF vary in range and severity from person to person. Symptoms you may experience include:

  • Constant coughing, wheezing and shortness of breath
  • High levels of salt in sweat; salty skin
  • Good appetite but poor growth/weight gain
  • Large, greasy bowel movements and long-lasting (chronic) diarrhea
  • Nasal polyps and chronic sinusitis
  • Chronic inflammation in the airways (bronchitis)
  • Frequent lung infections

We’re here to help.

Your CVS Specialty CF CareTeam is made up of pharmacists and nurses who are specially trained in caring for CF. They can help you manage your CF so you can get the best results.

Your CF CareTeam is ready to:

  • Answer all your questions
  • Work with your doctor
  • Provide your CF products, supplies and help to get started
  • Assist with questions about insurance and billing
  • Offer support and counseling on your CF and medication
  • Arrange delivery of your medications2

Your CareTeam provides specialized support so you can focus more on living your life, not your CF. Call your CareTeam at 1-866-845-6790.

This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about your medical condition and prior to starting any new treatment. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship, or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

1. Cystic Fibrosis Foundation® Website, Accessed August 2018.

2. Where allowed by law. In-store pick up is currently not available in Oklahoma. Puerto Rico requires first-fill prescriptions to be transmitted directly to the dispensing specialty pharmacy. Products are dispensed by CVS Specialty and certain services are only accessed by calling CVS Specialty directly. Certain specialty medication may not qualify. Services are also available at Long’s Drugs locations.