Cystic Fibrosis

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Basics

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

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• Living With
• Related Issues
• Genetics

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Research

• Clinical Trials
• Journal Articles

Resources

• Find an Expert

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• Children
• 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)
• Breakdown in Breathing: The Complexities of Cystic Fibrosis (National Institutes of Health)
• Cystic Fibrosis (American Lung Association)
• What is Cystic Fibrosis? (National Heart, Lung, and Blood Institute)

Symptoms

• What Are the Signs and Symptoms of 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)
• Cystic Fibrosis (CF): Chloride Sweat Test (Nemours Foundation) Also in Spanish
• Cystic Fibrosis: Prenatal Screening and Diagnosis (American College of Obstetricians and Gynecologists) Also in Spanish
• How Is Cystic Fibrosis Diagnosed? (National Heart, Lung, and Blood Institute)
• Newborn Screening for CF (Cystic Fibrosis Foundation)
• 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)
• How Is Cystic Fibrosis Treated? (National Heart, Lung, and Blood Institute)
• 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)
• Exercise and Cystic Fibrosis (CF) (Cystic Fibrosis Foundation) - PDF
• Living with Cystic Fibrosis (National Heart, Lung, and Blood Institute)
• Pulmonary Rehabilitation: MedlinePlus Health Topic (National Library of Medicine) Also in Spanish
• Supporting Nutrition: Understanding Tubefeeding (Cystic Fibrosis Foundation) - PDF

Related Issues

• Allergic Bronchopulmonary Aspergillosis (Cystic Fibrosis Foundation)
• CF and School (Cystic Fibrosis Foundation)
• Cystic Fibrosis-Related Diabetes (CFRD): Daily Management (Cystic Fibrosis Foundation) - PDF
• Germs and Staying Healthy (Cystic Fibrosis Foundation)
• Influenza (the Flu) (Cystic Fibrosis Foundation)
• Methicillin-Resistant Staphylococcus aureus (MRSA) (Cystic Fibrosis Foundation)

Genetics

• Genetics Home Reference: cystic fibrosis (National Library of Medicine)
• Learning about Cystic Fibrosis (National Human Genome Research Institute)
• What Causes Cystic Fibrosis? (National Heart, Lung, and Blood Institute)

Clinical Trials

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

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

• Article: Interventions for preventing and managing advanced liver disease in cystic...
• Article: Vitamin K supplementation for cystic fibrosis.
• Article: Palliative drug treatments for breathlessness in cystic fibrosis.
• 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

Children

• Cystic Fibrosis: Diet and Nutrition (Nemours Foundation)
• What's it Like to Have Cystic Fibrosis? (Nemours Foundation)

Teenagers

• Cystic Fibrosis: Diet and Nutrition (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