Facts About Cystic Fibrosis

Prepared by Cystic Fibrosis Research, Inc. (CFRI)

May 1995

WHAT IS CYSTIC FIBROSIS?

Cystic fibrosis (CF) is the most common, fatal hereditary disease in the U.S. CF is a disorder of the cells that line the lungs, small intestines, sweat glands and pancreas. Sticky, thick mucus contributes to the destruction of lung tissue and impedes gas exchange in the lungs. It also prevents nutrient absorption in the small intestines, and blocks ducts from the pancreas that release digestive enzymes. Approximately 85% of all people with CF cannot properly digest their food without supplemental enzymes.

People with CF exhibit some or all of these common symptoms, to varying degrees: salty-tasting skin excessive appetite along with poor weight gain loose, foul-smelling stools persistent cough, wheezing or pneumonia

Other symptoms may include the following: clubbed fingers digestive disorders smaller than average in height and weight susceptibility to heat prostration and dehydration male sterility diabetes and/or liver disorders

CF patients are frequently hospitalized for chronic lung infections and digestive disorders. People with CF must follow a strict regimen for the treatment of this disease. This regimen includes taking medications such as antibiotics to fight respiratory infections, enzymes to aid in food digestion, and vitamins to improve general health. Some people with CF use aerosols to open restricted airways. To dislodge mucus from the lungs, people with CF must undergo chest physical therapy or other therapy methods; in some cases, they must use oxygen. People with CF also must follow a strict diet, and avoid people with colds or infections.

HOW COMMON IS CF?

Anywhere from 20,000 to 30,000 American children and adults have CF. One in 2,500 American children is born with CF. Approximately 1,300 new cases are diagnosed each year. Improved diagnostic techniques have resulted in newly identified cases in people of all ages. Twelve million Americans, or 1 in 23, are symptomless carriers of the CF gene. At present, only half of those with CF survive to age 30.

HOW DO YOU KNOW IF YOU OR YOUR CHILD HAS CF?

Review the list of symptoms as described in this fact sheet. If you or your child has most of the symptoms indicated, you should ask your doctor about having a "sweat test." The sweat test is a reliable tool for diagnosing cystic fibrosis. This simple, painless test measures the amount of salt in the sweat. A high level of salt indicates that a person has CF. Recently developed genetic techniques using DNA analysis can be definitive when positive, but because hundreds of alleles can cause CF, no single genetic test works in all cases.

WHAT CAN YOU DO IF YOU HAVE CF?

Contact your physician immediately and follow the medical advice you are given. Become educated about the disease. Attend support groups, conferences, educational meetings, and read newsletters that are available through CFRI. Don't become discouraged. Many new advances in research provide hope for all who have this disease. Scientists have discovered the gene that causes CF and gene therapy research is moving forward. While there is no cure for CF at present, many recent developments in medicine (such as a new drug that reduces infections by breaking up thick mucus accumulations) make it easier for people to live their lives with this disease.

WHAT DOES CFRI DO ABOUT CF?

CFRI is an independent, nonprofit volunteer organization that is dedicated to saving the lives of children and adults with cystic fibrosis. We were founded in 1975 in order to fund life-saving research and sponsor educational programs. During our twenty year existence, CFRI has funded over $2.4 million toward cystic fibrosis research projects. Our educational program includes publishing a CFRI newsletter and educational brochures that are circulated to 13,000 readers throughout the world. We hold support meetings and monthly talks on CF research and living with CF. CFRI also sponsors an annual educational conference for the CF community as well as a summer retreat for young people with CF and their families.

HOW CAN YOU ASSIST CFRI?

Volunteer to help us raise funds for our research and educational programs. Participate in our major fundraising events, such as the annual golf tournament, or our Mother's Day Tea solicitation. Contribute funds to support the significant research that is taking place in universities and research centers throughout the United States. To find out how you can offer your much needed time and talents to CFRI, please contact us today.

Printed by CFRI as an educational community contribution. Information from this fact sheet may be reproduced for your use as long as you give credit for the information you've reproduced to CFRI.

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