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As yet, there is no known cure for CF, but there is real hope.

Comprehensive treatment programs have dramatically extended the life expectancy of persons with CF and many are living into their 20s, 30s and beyond.

As of 2001, the median age of survival of a person with cystic fibrosis is over 35.9 years of age. The median age of survival is the age beyond which half of the CF population can be expected to live.

Recent years have seen remarkable progress in CF research. Since 1989 when Canadian researchers discovered the gene responsible for CF, global research to find a cure for the disease has brought us closer and closer to a solution.

The astonishing pace of CF science suggests that there is good reason to feel optimistic about the future.

Prevention, Early Detection, and Treatment
Prevention of CF is not currently possible, except through targeted population screening for carrier status and genetic counseling about CF risk before a couple conceives.

Early detection can be accomplished with CF gene mutation testing prenatally, using amniocentesis or chorionic villi collection procedures. Newborn screening programs are starting to add IRT testing and CF gene mutation testing to their menus. Early identification of CF allows parents to get education, referral to a CF center for specialized help, and to start early treatments in their infants to minimize nutritional problems and lung damage.

Treatments are currently aimed at lessening symptoms and improving quality of life. Future research is exploring gene therapy as a way to replace the defective CF genes and looking for ways around the protein defect, such as finding other chloride channel regulators to compensate.

Treatments to ease symptoms include:

Medications: Aerosolized and inhaled medications are used to improve breathing, reduce respiratory swelling, and thin mucus. Oral, intravenous, and aerosolized antibiotics that can be inhaled are used to fight frequent respiratory infections.
Chest physiotherapy (CPT): Bronchial, or postural, drainage is performed a couple of times a day by placing the patient in a position that allows mucus to drain from the lungs. The chest or back is then clapped (percussed) to dislodge the mucus.
Exercise: Exercise helps loosen mucus and also stimulates coughing to aid in its removal.
Oral pancreatic enzymes, Vitamin A, D, E, and K supplements, and a diet low in fat, high in protein and high in calories help with gastrointestinal symptoms.
In vitro procedures in males with missing vas deferens aid in fertility.
Carefully monitored pregnancies are generally well tolerated in CF women with mild to moderate disease.
Lung transplants may be considered in some cases.

Cystic Fibrosis Redefined
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Cystic Fibrosis Signs and Symptoms CF interferes with electrolyte and fluid balances in the body. In most CF patients, sweat is up to five times saltier than normal. Losing excess sodium and chloride can affect the heart rhythm and may sometimes cause shock. In patients with CF, lubricating mucous in the lungs becomes thick and sticky, providing a breeding ground for microorganisms, causing frequent respiratory infections. These must be aggressively treated with intravenous, oral, and/or inhaled antibiotics. Much of the morbidity associated with CF is due to respiratory infections and lung complications. Mucous plugs in the pancreas lead to blocked ducts, resulting in vitamin deficiencies, malnutrition, and malabsorption because pancreatic enzymes are prevented from traveling to the intestines to digest protein. Oral pancreatic enzyme supplements and replacement of fat-soluble vitamins A, D, E and K can ease these symptoms, along with a low fat high protein diet. If pancreatic damage is sufficient, some CF patients eventually become diabetic. Other problems and symptoms associated with CF include: Meconium ileus (no stools in the first 24 to 48 hours of life) Weight loss, failure to thrive Pancreatitis; Delayed growth and an average 2-year delay in sexual development at puberty Chronic diarrhea and foul-smelling, greasy stools Growths (polyps) in the nasal passages Enlargement or rounding (clubbing) of the fingertips and toes due to a chronic reduced oxygen environment from lung involvement Coughing or wheezing, thick sputum Biliary cirrhosis caused by blocked bile ducts in the liver Rectal prolapse (protrusion of the rectum through the anus)
How is CF diagnosed? If a physician suspects CF, he or she will probably suggest a "sweat test". This simple and painless test measures the amount of salt in the sweat. A high salt level, along with other symptoms, points to the presence of cystic fibrosis. Gentic tests are also being used to diagnose the disease. Prenatal genetic testing by amniocentesis or chorionic villus sampling (CVS), or genetic testing with a blood sample or a swab of cheek cells that can be analyzed to confirm the presence or absence of common CF mutations. Is There a Cure for Cystic Fibrosis? As yet, there is no known cure for CF, but there is real hope. Comprehensive treatment programs have dramatically extended the life expectancy of persons with CF and many are living into their 20s, 30s and beyond. As of 2001, the median age of survival of a person with cystic fibrosis is over 35.9 years of age. The median age of survival is the age beyond which half of the CF population can be expected to live. Recent years have seen remarkable progress in CF research. Since 1989 when Canadian researchers discovered the gene responsible for CF, global research to find a cure for the disease has brought us closer and closer to a solution. The astonishing pace of CF science suggests that there is good reason to feel optimistic about the future. Prevention, Early Detection, and Treatment Prevention of CF is not currently possible, except through targeted population screening for carrier status and genetic counseling about CF risk before a couple conceives. Early detection can be accomplished with CF gene mutation testing prenatally, using amniocentesis or chorionic villi collection procedures. Newborn screening programs are starting to add IRT testing and CF gene mutation testing to their menus. Early identification of CF allows parents to get education, referral to a CF center for specialized help, and to start early treatments in their infants to minimize nutritional problems and lung damage. Treatments are currently aimed at lessening symptoms and improving quality of life. Future research is exploring gene therapy as a way to replace the defective CF genes and looking for ways around the protein defect, such as finding other chloride channel regulators to compensate. Treatments to ease symptoms include: Medications: Aerosolized and inhaled medications are used to improve breathing, reduce respiratory swelling, and thin mucus. Oral, intravenous, and aerosolized antibiotics that can be inhaled are used to fight frequent respiratory infections. Chest physiotherapy (CPT): Bronchial, or postural, drainage is performed a couple of times a day by placing the patient in a position that allows mucus to drain from the lungs. The chest or back is then clapped (percussed) to dislodge the mucus. Exercise: Exercise helps loosen mucus and also stimulates coughing to aid in its removal. Oral pancreatic enzymes, Vitamin A, D, E, and K supplements, and a diet low in fat, high in protein and high in calories help with gastrointestinal symptoms. In vitro procedures in males with missing vas deferens aid in fertility. Carefully monitored pregnancies are generally well tolerated in CF women with mild to moderate disease. Lung transplants may be considered in some cases. http://www.labtestsonline.org/index.html http://www.cysticfibrosis.ca/