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Cystic Fibrosis Links
Cystic Fibrosis Foundation
Cystic Fibrosis Research, Inc.
CareFirst for Life is a comprehensive support program for patients with cystic fibrosis, family members, and caregivers. The CareFirst for Life Program is free for patients taking ULTRASE/ULTRASE MT capsules and is easy to use. It offers many resources that encourage patients to keep up with their treatments through valuable information and interactive tools. As a member, you can count on continuous educational support, numerous product offerings (including vitamins and supplements), and additional benefits.
There is substantial research showing that the good management of stress, anxiety, anger, grief, depression and other personal and family dynamics can improve health outcomes, as well as other aspects of the quality of life. This information is important in the care of chronic illnesses. This is particularly true in CF as the medical successes increase the number of adult patients looking to lead independent and productive lives. The one page papers listed on this site have been created to provide easy to read information pages with supporting references, that demonstrate the connections between psychological, social and spiritual well-being, and physical health.
Video of Airway Clearance Techniques
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Cystic Fibrosis F.A.Q.
How should I order my treatments? CFF Guidelines and our center staff recommend the following sequence for inhaled medications:
Bronchodilators:
(albuterol, Combivent, Xopenex) to open the airways
Hypertonic Saline (%7):
to mobilize mucus and induce airway clearance
Pulmozyme (DNAse):
to thin mucus (Note: studies find no significant differences for order of inhaling DNAse, hence patient preference can govern timing)
Airway Clearance Technique:
Vest, Flutter, chest PT, IPPV, etc.
Antibiotics:
(TOBI, colistin) the previous therapies open and clear the airways of mucus, thereby improving the distribution and therapeutic impact of these medications
Steroids:
(Flovent, Pulmicort, QVAR) What should I do if I cough up blood? If you start coughing up blood, stop Pulmozyme, saline, airway clearance technique, and inhaled antibiotics. Call your CF doctor or nurse for further advice. What do I do if I get a cold and have respiratory symptoms? Follow your sick plan. Every CF patient has a well and sick plan. If you need one call the center now. Don't ever be without one.
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Cystic Fibrosis
Introduction to Cystic Fibrosis
Cystic Fibrosis (CF) is a genetic disease affecting approximately 30,000 children and adults in the United States. A defective gene causes the body to produce abnormally thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections. These thick secretions also obstruct the pancreas, preventing digestive enzymes from reaching the intestines to help break down and absorb food. The mucus also can block the bile duct in the liver, eventually causing permanent liver damage in approximately six percent of people with CF.
More than 10 million Americans are unknowing, symptomless carriers of the defective CF gene. An individual must inherit two defective CF genes—one from each parent—to have CF. Each time two carriers conceive, there is a 25 percent chance that their child will have CF; a 50 percent chance that the child will be a carrier of the CF gene; and a 25 percent chance that the child will be a non-carrier.
CF occurs in approximately one of every 3,500 live births. About 1,000 new cases of CF are diagnosed each year. More than 80 percent of patients are diagnosed by age three; however, nearly 10 percent of newly diagnosed cases are age 18 or older.
People with CF can have a variety of symptoms including: very salty-tasting skin; persistent coughing, at times with phlegm; wheezing or shortness of breath; an excessive appetite but poor weight gain; and greasy, bulky stools. Symptoms vary from person to person due, in part, to the more than 1,000 different mutations that can occur in the CF gene.
Living with Cystic Fibrosis
The sweat test is the standard diagnostic test for CF. A sweat test should be performed at a CF Foundation-accredited care center where strict guidelines are followed to ensure accurate results. This simple and painless procedure measures the amount of salt in the sweat. A high salt level indicates CF.
The treatment of CF depends upon the stage of the disease and the organs involved. Clearing mucus from the lungs is an important part of the daily CF treatment regimen. Chest physical therapy is a form of airway clearance done by vigorous clapping on the back and chest to dislodge the thick mucus from the lungs. Other types of treatments include TOBI© (tobramycin solution for inhalation), an aerosolized antibiotic used to treat lung infections; Pulmozyme©, a mucus-thinning drug shown to reduce the number of lung infections and improve lung function; and azithromycin, an antibiotic recently proven to be effective in people with CF whose lungs are chronically infected with the common Pseudomonas aeruginosa bacteria. In addition, approximately 90 percent of all people with CF take pancreatic enzyme supplements to help them absorb food in digestion.
According to the CF Foundation's National Patient Registry, the median age of survival for a person with CF is 37 years. As more advances have been made in the treatment of CF, the number of adults with CF has steadily grown. Today, nearly 40 percent of the CF population is age 18 and older. Adults, however, may experience additional health challenges including CF-related diabetes and osteoporosis. CF also can cause reproductive problems - more than 95 percent of men with CF are sterile. But, with new technologies, some are becoming fathers. Although many women with CF are able to conceive, limited lung function and other health factors may make it difficult to carry a child to term.
There are more than 117 CF Foundation-accredited care centers across the United States that specialize in the diagnosis of CF and provide care to people with the disease. Care center staff include physicians, nurses, nutritionists, respiratory therapists, social workers, genetics counselors, and other medical professionals.
BAPP CF Resources
The following are intended to help organize and supplement the CF patient's, family's and caregivers' knowledge about CF. The information in these binders may change as we learn more about CF. Please speak to your CF doctor about any new information or changes.CF Team.
- Team CF: Targeting Enhanced Annual Measurements
- Moving on... Transitioning from the Pediatric to the Adult Care Team
Links coming soon
Cystic Fibrosis Foundation
The mission of the Cystic Fibrosis Foundation, a nonprofit donor-supported organization, is to assure the development of the means to cure and control cystic fibrosis and to improve the quality of life for those with the disease.
The Foundation is the leading organization in the United States devoted to cystic fibrosis. It funds and accredits more than 115 CF care centers, 95 adult care programs and 50 affiliate programs, and more than 75 chapters and branch offices nationwide.
The Cystic Fibrosis Foundation is one of the most efficient organizations of its kind. It has received a four-star rating for sound fiscal management from Charity Navigator, the largest independent charity evaluator in the United States and is an accredited charity of the Better Business Bureau's Wise Giving Alliance.
The Foundation's business model has been recognized by the National Institutes of Health and by publications such as Forbes, The New York Times, The Wall Street Journal and BusinessWeek.
CysticFibrosis.com
Check out the latest blogs, forums and videos from CysticFibrosis.com. This site has hundreds of educational and community resources for CF patients, families and friends. However, nothing on the site is intended to be instructional for medical diagnosis or treatment.
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