Cystic Fibrosis
What is Cystic Fibrosis?
Cystic Fibrosis (CF) is the most common, fatal hereditary disease in the United States.
Cystic Fibrosis (CF) affects approximately 30,000 children and young adults in the United States. CF causes a sticky, thick mucus to line the lungs, small intestines, sweat glands and pancreas. This results in the deterioration of lung tissue making it difficult to breathe. It also prevents necessary nutrient absorption in the small intestine and blocks pancreatic ducts from releasing digestive enzymes. 1 in every 23 Americans are symptomless carriers of the CF gene.
Cystic Fibrosis is an autosomal recessive genetic disease. This means that to have the disease, a child must inherit two copies of the defective gene, one from each parent. Approximately 1 in 25 Americans carries a single cystic fibrosis gene, and they do not experience any significant health problems. This means that potentially many adults may not know that they carry the gene.
CF patients are frequently hospitalized for chronic lung infections and digestive disorders. A typical hospital stay is 10 to 14 days.
Symptoms
CF patients exhibit some of all of these symptoms to varying degrees:
- 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 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 cold 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.
How do you know if you or your child has CF?
If you believe your child may have CF, you should ask your doctor about having a "sweat test". The Sweat Chloride Test is a reliable diagnostic tool. This simple, painless test measures the amount of salt in the sweat. A high level of salt may indicate the prescence of CF.
What can you do if you have CF?
Work closely with your physician for proper treatment.
Educate yourself about the disease. Utilize the Lungs for Life Foundation website as your resource and link to the many CF support groups, conferences and newsletters available.
While there is no cure for CF at present, do not become discouraged! There are many excellent treatment options and millions of dollars are spent each year in the search for a cure.
Resource Links:
Cystic Fibrosis Foundation
www.cff.org
(800) FIGHT CF (344-4823)
CF Pharmacy of Florida
www.mycfpharmacy.com
800-741-4427
Genentech Access Solutions: Pulmozyme Inhalation Solution
www.genentech-access.com/pulmozyme/patients
1-866-4ACCESS
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