Cystic Fibrosis

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Basics

• Summary
• Start Here
• Diagnosis and Tests
• Prevention and Risk Factors
• Treatments and Therapies

Learn More

• Living With
• Related Issues
• Genetics

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Research

• Clinical Trials
• Journal Articles

Resources

• Find an Expert

For You

• Teenagers
• Patient Handouts

Summary

Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs. CF causes your mucus to be thick and sticky. The mucus clogs the lungs, causing breathing problems and making it easy for bacteria to grow. This can lead to repeated lung infections and lung damage.

The symptoms and severity of CF can vary. Some people have serious problems from birth. Others have a milder version of the disease that doesn't show up until they are teens or young adults. Sometimes you will have few symptoms, but later you may have more symptoms.

CF is diagnosed through various tests, such as gene, blood, and sweat tests. There is no cure for CF, but treatments have improved greatly in recent years. In the past, most deaths from CF were in children and teenagers. Today, with improved treatments, some people who have CF are living into their forties, fifties, or older. Treatments may include chest physical therapy, nutritional and respiratory therapies, medicines, and exercise.

NIH: National Heart, Lung, and Blood Institute

Start Here

• About Cystic Fibrosis (Cystic Fibrosis Foundation)
• Cystic Fibrosis (American Lung Association)
• What Is Cystic Fibrosis? (National Heart, Lung, and Blood Institute)

Diagnosis and Tests

• CF Mutation Panel (American Association for Clinical Chemistry)
• Chymotrypsin Test (American Association for Clinical Chemistry)
• Cystic Fibrosis (CF) Respiratory Screen: Sputum (Nemours Foundation)
• Newborn Screening for CF (Cystic Fibrosis Foundation)
• Stool Elastase (National Library of Medicine) Also in Spanish
• Sweat Test for Cystic Fibrosis (National Library of Medicine) Also in Spanish
• Trypsinogen Test (American Association for Clinical Chemistry)

Prevention and Risk Factors

• Cystic Fibrosis and Pregnancy (March of Dimes Birth Defects Foundation) Also in Spanish

Treatments and Therapies

• Drug Development Pipeline (Cystic Fibrosis Foundation)
• Treatments and Therapies for Cystic Fibrosis (Cystic Fibrosis Foundation)

Living With

• Airway Clearance Techniques (ACTs) (Cystic Fibrosis Foundation)
• Basics of Postural Drainage and Percussion (Cystic Fibrosis Foundation)
• Day-to-Day Exercise and Cystic Fibrosis (CF) (Cystic Fibrosis Foundation) - PDF
• Dietary Tips for Kids With Cystic Fibrosis (Nemours Foundation)
• Pulmonary Rehabilitation: MedlinePlus Health Topic (National Library of Medicine) Also in Spanish
• Supporting Nutrition: Understanding Tubefeeding (Cystic Fibrosis Foundation) - PDF

Related Issues

• Aspergillus and Allergic Bronchopulmonary Aspergillosis (Cystic Fibrosis Foundation)
• CF and School (Cystic Fibrosis Foundation)
• COVID-19: COPD, Cystic Fibrosis, and Pulmonary Fibrosis (Centers for Disease Control and Prevention) Also in Spanish
• Cystic Fibrosis-Related Diabetes (CFRD): Daily Management (Cystic Fibrosis Foundation) - PDF
• Germs and Staying Healthy (Cystic Fibrosis Foundation)
• Marvels of Mucus and Phlegm: The Slime That Keeps You Healthy (National Institutes of Health) Also in Spanish
• Methicillin-Resistant Staphylococcus aureus (MRSA) (Cystic Fibrosis Foundation)

Genetics

• Cystic fibrosis: MedlinePlus Genetics (National Library of Medicine)
• Learning about Cystic Fibrosis (National Human Genome Research Institute)

Clinical Trials

• ClinicalTrials.gov: Cystic Fibrosis (National Institutes of Health)

Journal Articles References and abstracts from MEDLINE/PubMed (National Library of Medicine)

• Article: IRE1α Is a Therapeutic Target for Cystic Fibrosis Airway Inflammation.
• Article: Functional and Transcriptional Adaptations of Blood Monocytes Recruited to the Cystic...
• Article: Is the ENaC Dysregulation in CF an Effect of Protein-Lipid Interaction...
• Cystic Fibrosis -- see more articles

Find an Expert

• Cystic Fibrosis Foundation
• National Heart, Lung, and Blood Institute
• National Institute of Diabetes and Digestive and Kidney Diseases

Teenagers

• Dietary Tips for Teens With Cystic Fibrosis (Nemours Foundation)

Patient Handouts

• Cystic fibrosis (Medical Encyclopedia) Also in Spanish
• Cystic fibrosis - nutritional considerations (Medical Encyclopedia) Also in Spanish
• How to breathe when you are short of breath (Medical Encyclopedia) Also in Spanish
• Neonatal cystic fibrosis screening (Medical Encyclopedia) Also in Spanish
• Postural drainage (Medical Encyclopedia) Also in Spanish
• Sweat electrolytes test (Medical Encyclopedia) Also in Spanish