Cystic fibrosis (CF) is a fatal genetic disease affecting over 30,000 Americans today.  An additional 12 million Americans - approximately one in 23, unknowingly carry only one gene and are unaffected by the disease.
To have the disease, a child must inherit a defective copy of the CF gene from each parent. When two carriers conceive, there is a 25 percent chanc that their child will have CF, a 50 percent chance they will be a carrier and a 25 percent chance the child will be a non-carrier.

How does CF affect the body?

CF is a "multi-system" disease, meaning that it affects many body organs.  However, most of the symptoms have to do with the lungs and digestion. CF causes a defect in one of the body’s chromosomes leading to the production of abnormally thick mucus.  This sticky mucus primarily affects the lungs, leading to an increased incidence of lung infections, (the sticky mucus provides an ideal environment for bacterial growth) ultimately leading to irreversible lung tissue damage, and the pancreas, blocking the release of it’s enzymes to digest food, leading to malabsorption and malnutrition.

Other illnesses associated with CF:

- Intestinal obstruction
- Diabetes
- Liver disease
- Infertility in men and fertility problems in women
- Clubbed fingers and toes
- Small growths (polyps) in the nose

How is CF Treated?

People with CF should be made to eat a high calorie diet rich in fatty and sugary foods. Because of the malabsorption their energy intake requirements are much higher than those of typical children. Good nutrition is essential to maintain a person’s optimum health. With each meal or snack, most people with CF need to take capsules that supply the missing pancreatic enzymes and allow proper digestion.

Thirty years ago children with CF died by the age of 10 but today with the help of antibiotics, nebulizer treatments, daily chest physiotherapy, (this involves vigorous massage to help loosen the sticky mucus), vaccinations against influenza and pneumonia and the use of digestive enzymes, the life expectancy of people diagnosed with CF has increased to a median age of 37.4 years.  Many CF patients in the most critical stage of the disease are on waiting lists for lung transplants. To find out more about organ donation and how you can become an organ donor, Click the Donate Life icon on right.

Thanks, in large part, to the generous donations of CF family and friends, there are treatments being developed that may dramatically improve the everyday lives of CF patients being done by the Cystic Fibrosis Foundation.  

To name one in particular, VX-770 by Vertex Pharmaceuticals, has been shown to decrease chloride levels in sweat tests thereby being the only treatment proven to work directly on the chloride channel which is the basic defect in CF patients. Not since the discovery of the gene in 1987 have things been so exciting!

We at the CRCFF hope that this research being done will be beneficial to ALL CF patients and we will be following its development closely. Vertex Pharmaceuticals also has another drug similar to VX-770 that targets a very common gene defect  in CF patients named Delta508 which is also coming down the drug research pipeline and may be available to CF patients in the following few years.