CHARLOTTE CF FAMILIES
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What is...

These topics are designed to help you understand Cystic Fibrosis terminology. We also want to help you and your child know what to expect during a procedure, in clinic, or at the hospital. We hope this knowledge helps you feel more prepared.
Cystic Fibrosis

Cystic Fibrosis

Cystic fibrosis is a progressive, genetic disease that causes persistent lung infections and limits the ability to breathe over time.

In people with CF, mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause the CFTR protein to become dysfunctional. When the protein is not working correctly, it’s unable to help move chloride — a component of salt — to the cell surface. Without the chloride to attract water to the cell surface, the mucus in various organs becomes thick and sticky.
In the lungs, the mucus clogs the airways and traps germs, like bacteria, leading to infections, inflammation, respiratory failure, and other complications. For this reason, minimizing contact with germs is a top concern for people with CF.

In the pancreas, the buildup of mucus prevents the release of digestive enzymes that help the body absorb food and key nutrients, resulting in malnutrition and poor growth. In the liver, the thick mucus can block the bile duct, causing liver disease. In men, CF can affect their ability to have children.

Symptoms of CF

People with CF can have a variety of symptoms, including:
  • Very salty-tasting skin 
  • Persistent coughing, at times with phlegm
  • Frequent lung infections including pneumonia or bronchitis
  • Wheezing or shortness of breath
  • Poor growth or weight gain in spite of a good appetite
  • Frequent greasy, bulky stools or difficulty with bowel movements
LEARN MORE

Intro to CF
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  • HOME
  • ABOUT
    • MEET THE CF CARE TEAM
    • CONTACT
  • RESOURCES
    • COVID-19 Mental Health Resources
    • Virtual Care
    • Programs & Opportunities for CF Families
    • Financial Assistance
    • Support Groups
    • Mental Health
    • Legal Aid
    • Nutrition & Supplements
    • Newsletter
  • WHAT IS...
    • Cystic Fibrosis
    • Antibiotic Resistance
    • Bronchodilator
    • Chest X-Ray
    • Hypertonic Saline
    • Inhaled Antibiotics
    • Labs / Blood Work
    • Mucus Thinners
    • Oral Glucose Tolerance Test (OGTT)
    • PEP Device
    • Pulmonary Function Test (PFT)
    • Pulmozyme / Dornase Alfa
    • Sputum Cultures
    • Sweat Test
  • EVENTS
    • 2022 CF Education Day Recap
    • 2021 CF Education Day Recap
    • 2020 CF Education Day Recap
  • NEWS & UPDATES