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Cystic Fibrosis

Home > Illnesses and Disabilities Home > Types of Illnesses & Disabilities > Cystic Fibrosis

Cystic fibrosis (CF) is a disease caused by a defect in a gene that helps produce mucus. Mucus is the fluid that coats the lining of airways and other passageways in your body. Mucus prevents these passageways from drying out or becoming infected. Normally, mucus is watery. But CF causes the mucus to be thick and sticky. This type of mucus clogs the lungs, making it hard to breathe. It also makes it easy for germs to infect your lungs. Over time, these infections can damage your lungs.

The thick, sticky mucus can also make it hard for your small intestine to break down the food that you eat. As a result, many of the nutrients in your food are not absorbed and leave your body, and you can become malnourished.

The most common symptoms of CF include:

• frequent coughing that brings up thick mucus
• frequent lung infections (such as bronchitis and pneumonia)
• salty-tasting skin (because the sweat glands produce sweat that is high in salt)
• diarrhea or bulky, foul-smelling, and greasy stools
• huge appetite but poor weight gain
• stomach pain and discomfort caused by too much gas in your intestines

The main treatments for lung problems in people with CF are:

• antibiotics for infections of the airways
• chest physical therapy. This involves pounding your chest and back over and over again to dislodge the mucus from your lungs so that you can cough it up.
• being physically active. This helps loosen the mucus and encourages coughing to clear the mucus.
• other medicines, such as mucus-thinning drugs

The treatments for digestive problems in people with CF include:

• eating a well-balanced, high-calorie diet
• medicines to help break down food

Advances in medical research may in time lead to a cure for this disease. One promising approach is gene therapy, in which the defective CF gene is replaced with a normal gene.

Additional Resources

Publications

1.   Employees with Respiratory Impairments - This publication for employers contains basic information regarding questions that might be considered when determining accommodations for individuals with respiratory impairments.

   http://www.jan.wvu.edu/media/respiratory.html

2.   What Is Cystic Fibrosis? - This publication describes the risk factors for cystic fibrosis, diagnosis, treatment, and other important information.

   http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html

3. Cystic Fibrosis - What is it? (Copyright © Dolan DNA Learning Center) - This interactive web site contains tutorials about cystic fibrosis, what causes it, how it is treated, what it is like to have it, and more.

   http://www.yourgenesyourhealth.org/cf/whatisit.htm

4.   JAMA Patient Page: Cystic Fibrosis (Copyright © JAMA) - This fact sheet contains information for patients with cystic fibrosis. It discusses the signs and symptoms, ways to manage the disease and how it is inherited.

   http://jama.ama-assn.org/cgi/reprint/284/14/1884.pdf

Organizations

1.   National Heart, Lung, and Blood Institute Information Center, NHLBI, NIH, HHS
2. Boomer Esiason Foundation
3. Cystic Fibrosis Foundation

= Indicates Federal Resources

Content last updated May 15, 2008.