Cystic Fibrosis Information

 
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About Cystic Fibrosis

Life Expectancy Improvements

Many of you, either through your experiences with us over the past few years or with other friends or family affected by this disease, are familiar with what cystic fibrosis is. For those that aren't, here's a brief synopsis:

Cystic fibrosis is a life-threatening genetic disorder that affects over 30,000 Americans. This disease is marked by chronic infections, clogged airways, and digestive and reproductive problems.

In cystic fibrosis patients, problems occur because the mucus in their lungs is very thick and sticky. Instead of serving as a lubricant, it clogs the respiratory system and allows bacteria to grow within it, impairing the body's natural defenses. The thick mucus also obstructs the pancreas, preventing enzymes from reaching the intestines to digest food.

Cystic fibrosis occurs in roughly one of every 3,500 live births. More than 10 million Americans are unknowing, symptomless carriers of the disease. To contract it, a child must inherit a defective copy of the CF gene from each parent. 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.

People with CF 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 mutations of the CF gene.

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.

Although cystic fibrosis is not yet curable, the good news is that the situation is not hopeless. Each day since the cystic fibrosis gene was discovered in 1989, the pace of CF research has greatly accelerated, and scientists continue to find ways to help those who live with the disease. These treatments include the use of tobramycin - a drug therapy for CF approved by the Food and Drug Administration in 1997 - and experimental gene therapy treatment.

-- Cystic Fibrosis Information from www.cff.org

About the Cystic Fibrosis Foundation

Hannah doing a daily breathing treatment -- December, 2012.
Hannah doing a daily breathing
treatment -- December, 2012.
The Cystic Fibrosis Foundation's mission since its inception in 1955 has been 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 CF Foundation remains one of the top voluntary health organizations in the country at efficiently using its funds raised to invest in medical and research programs. SmartMoney magazine has cited the CF Foundation as a charity that "won't waste your money." We encourage you to support the Cystic Fibrosis Foundation through donations and participation in CFF fundraising activities.

For more information about cystic fibrosis and the efforts to find a cure, we recommend two web sites very highly:

  • www.esiason.org - Boomer Esiason, former NFL quarterback, started the Boomer Esiason Foundation when his young son, Gunnar, was diagnosed with cystic fibrosis. The purpose of the organization is to raise awareness for the disease and to make sure that researchers and scientists have the resources they need to accelerate their efforts.
  • www.cff.org - The Cystic Fibrosis Foundation assures the development of the means to cure and control cystic fibrosis and to improve the quality of life for those with the disease. They are also a tremendous resource of reliable information about cystic fibrosis.

 We leave you with a quote from Robert Beall, president and chief executive officer of the Cystic Fibrosis Foundation:

“CF is no longer a death sentence. Today, the median age of CF patients is over 37, and more than 45% of our patients are 18 and older.”

These are very encouraging words, but it's not enough. Please help us help those combating CF to find a cure for Hannah and others like her.