About CF

Cystic Fibrosis is…

…a progressive, genetic (inherited) disease affecting well over 30,000 Americans. It causes the body to produce abnormally thick and sticky mucous that affects the entire body. CF can (and most often will) lead to lung and sinus disease, wreak havoc with the gastrointestinal system (including the pancreas and liver), cause poor growth (due to lack of absorbtion in the GI tract), and even affect fertility. Damage to the pancreas can also cause something known as CFRD ~ Cystic Fibrosis Related Diabetes. Though mostly known for the complications it causes the lungs, many of the other symptoms can be just as severe. (Click herefor more in-depth info.)

A child born to a couple who are both carriers the defective CF gene has a 25% chance of having Cystic Fibrosis; a 50% chance of being a carrier of the defective gene (but not have CF) and a 25% chance of being neither a carrier nor having CF. It is estimated that approximately 1 in 31 Americans are symptomless carriers of this defective gene.

Some symptoms of CF can include:

~ very salty-tasting skin

~ difficulty gaining weight despite a large appetite

~ bulky, frequent, greasy, and foul-smelling stools

~ sinus problems (polyps and infections)

~ coughing, breathing troubles, repeated pneumonias, etc.

While these are general symptoms, there are many different ways CF can manifest itself within the body. There are also numerous different mutations and combinations of the CF gene that provide for a varying degree of symptom occurences and levels of severity.

Most people living with CF need to take supplemental digestive enzymes to help them digest and properly the absorb the fat and nutrients from their food; the people in this group are considered to be pancreatic insufficient. A much smaller minority of CF patients do not require the supplemental enzymes; they are considered to be pancreatic sufficient.

Daily airway clearance techniques (including nebulizer and inhaler treatments) are necessary to help keep the lungs clear of the sticky mucous.

Although there are a wide variety of medical therapies available today that help to provide some control of some aspects of the disease, none of them are cures. However, it does give us hope that even more effective treatments will eventually be discovered to continue to shape the way we treat the disease ~ and to continue to improve the quality of life for those living with it. Lung transplants (as well as liver and other organs) are not the answer; they are a bandaid used as an attempt to prolong (and improve the quality of) life and are not very sucessful for so many. Many more die waiting for a transplant. There are some inspirational success stories, but unfortunately those are few and far between. That said, please consider registering as organ donor; you can be someone’s hero.

The current median age for someone with CF (as reported by the Cystic Fibrosis Foundation) is around 37 years. While that is phenomenal progress, we still need to recognize that HALF of all CF patients do not make it that far and we are losing young people every day.