| Cystic Fibrosis Information |
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What Is Cystic Fibrosis?
Cystic Fibrosis (CF) is the most common, fatal hereditary disease in the U.S. (Source:
CF is a cell disorder that creates mucus that lines the lungs, small intestines, sweat glands and pancreas. This thick, sticky mucus
contributes to the destruction of lung tissue and impedes necessary gas exchange in the lungs. It also prevents nutrient absorption
in the small intestines, and blocks pancreatic ducts from releasing digestive enzymes. Approximately 85% of all people with CF
cannot properly digest their food without supplemental enzymes.
Cystic Fibrosis (CF) is the most common, fatal hereditary disease in the U.S. (Source:
CF is a cell disorder that creates mucus that lines the lungs, small intestines, sweat glands and pancreas. This thick, sticky mucus
contributes to the destruction of lung tissue and impedes necessary gas exchange in the lungs. It also prevents nutrient absorption
in the small intestines, and blocks pancreatic ducts from releasing digestive enzymes. Approximately 85% of all people with CF
cannot properly digest their food without supplemental enzymes.
CF patients exhibit some or all of these symptoms to varying degrees:
CF patients are frequently hospitalized for chronic lung infections and digestive disorders. A typical hospital stay is 10 to 14 days.
How Is Cystic Fibrosis Treated?
CF patients follow a strict regimen to treat the disease as well as to reduce the risk of contracting outside infections. For example,
patients must avoid others with colds or other respiratory infections. The treatment regimen can include:
Many CF patients in the most critical stage of the disease are on waiting lists for lung transplants. The wait can be up to 2 years at
some transplant centers.
How Common Is CF?
· 30,000 American Children and Adults have CF. (This may seem like a small number but only half of those with CF survive to age
31.)
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
Chloride 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 may indicate the presence of CF. Recently developed genetic techniques using DNA analysis can be definitive
when positive, but because hundreds of alleles (or groups of genes) can cause CF, no single genetic test works in all cases.
What Can You Do If You Have CF?
- Salty-tasting skin
- Excessive appetite along with poor weight gain
- Persistent cough, wheezing or pneumonia
- Loose, foul smelling stools
- Clubbed fingers
- Digestive disorders
- Smaller than average height and weight
- Susceptibility to heat prostration and dehydration
- Male sterility
- Asthma
- Diabetes and/or liver disorders
CF patients are frequently hospitalized for chronic lung infections and digestive disorders. A typical hospital stay is 10 to 14 days.
How Is Cystic Fibrosis Treated?
CF patients follow a strict regimen to treat the disease as well as to reduce the risk of contracting outside infections. For example,
patients must avoid others with colds or other respiratory infections. The treatment regimen can include:
- Oral and/or IV Antibiotics to fight respiratory infections.
- Vitamins such as Vitamin E, C, Zinc, Calcium, and a Multi-Vitamin to improve general health.
- Chest physical therapy, bronchodilators, and other treatments that clear airways and dislodge mucus from the lungs (may be
required as many as four times a day). Bronchodilators are generally given in the form of aerosol treatments, but the same
medication can also be administered by mouth. - Steroids, such as prednisone, to reduce inflammation in the lungs.
- Supplemental oxygen therapy to relieve the lungs and the body's struggle to oxygenate the blood.
- Enzymes to aid in digestion.
- Feeding tubes administered during the night with calorically dense liquid nutrition, for weight gain.
Many CF patients in the most critical stage of the disease are on waiting lists for lung transplants. The wait can be up to 2 years at
some transplant centers.
How Common Is CF?
· 30,000 American Children and Adults have CF. (This may seem like a small number but only half of those with CF survive to age
31.)
- One in 2,500 American children are 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 (12,000,000) Americans or 1 in 23 carry the CF gene but have no symptoms. This is a critical factor in any
hereditary disease.
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
Chloride 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 may indicate the presence of CF. Recently developed genetic techniques using DNA analysis can be definitive
when positive, but because hundreds of alleles (or groups of genes) can cause CF, no single genetic test works in all cases.
What Can You Do If You Have CF?
- Contact your physician immediately.
- Educate yourself about the disease. There are many CF support groups, conferences, educational meetings and newsletters
with information. There is also a great deal of information on the internet. For information about CF related resources contact
the Cystic Fibrosis-Reaching Out Foundation.
- 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 to manage the disease.